Treatment of persistent post-adenoidectomy velopharyngeal insufficiency by sphincter pharyngoplasty

Objective

Persistent hypernasality after adenoidectomy is an infrequent problem in children with normal palate. However if it happened, it can render a child's speech unintelligible resulting in serious affection of social life. We aimed in this study to identify the causes of persistent post-adenoidectomy velopharyngeal insufficiency and to assess the efficacy of sphincter pharyngoplasty in the treatment of such problem.

Methods

This study was conducted on 18 patients complained of hypernasal speech following removal of their adenoids after variable periods of failed expected spontaneous improvement. Their hypernasality was rated as being mild, moderate and severe, all cases were subjected to conservative treatment in the form of speech therapy for 3 months to correct the problem, and patients that did not respond to speech therapy were subjected to surgical intervention in the form of sphincter pharyngoplasty. Velopharyngeal closure was assessed using flexible nasopharyngoscopy, while speech was assessed using auditory perceptual assessment and nasometry.

Results

Hypernasality was mild in 9 cases, moderate in 7 cases and severe in 2 cases. Flexible nasopharyngocopy showed occult submucous cleft in 5 cases, short palate in 2 cases, and deep nasopharynx in 3 cases. Speech improvement was achieved in 8 cases after completion of speech therapy program (all had mild hypernasality with no anatomical palatal defects). Ten patients that had palatal defects were subjected to sphincter pharyngoplasty, 8 of them showed complete recovery, while 2 cases with severe hypernasality showed partial improvement of their speech.

Conclusions

Persistent post-adenoidectomy velopharyngeal insufficiency may be due to anatomical abnormalities of the palate such as an occult submucous cleft, short palate or deep nasopharynx; such conditions may be overlooked during the preoperative preparation for adenoid removal. Speech therapy is an effective method in mild hypernasality especially if there is no anatomical abnormality, while surgical correction is usually needed in moderate and severe cases, and sphincter pharyngoplasty is a useful choice for those patients.     

Furlow technique for treatment of soft palate fistula

Objective

Fistula of the palate is a common complication of palatoplasty, it leads to nasal regurgitation of fluids and hypernasality of speech. Its treatment is technically difficult due to paucity and fibrosis of palatal tissues. The aim of this study was to evaluate the efficacy of closure of soft palate fistula by using Furlow double opposing Z-palatoplasty.

Methods

Nineteen patients were subjected for repair of their soft palate fistulas using Furlow Z-plasty. Pre and postoperative speech analysis using auditory perceptual assessment, measurement of nasalance score using nasometric assessment, and measurement of velar movement using flexible nasopharyngoscopy were done.

Results

All cases showed complete closure of their fistulas at first attempt, with no operative or postoperative complications. Recurrence was not recorded in any case after a follow up period of at least 12 months. Significant improvement of speech quality and nasalance score was achieved. Flexible nasopharyngoscopy showed postoperative increase in velar movement which was not significant relative to the preoperative records.

Conclusions

Treatment of soft palate fistula by using Furlow technique is an effective method as a primary treatment with a high success rate and a good functional outcome.     

A novel technique for superior-based pharyngeal flaps: 10-year results with formal speech outcomes assessment

Purpose

Describe a novel technique for superior-based pharyngeal flaps allowing restoration of bulk to the soft palate and intraoperative fine-tuning of lateral port size, while avoiding midline palate-splitting. Validated speech assessment tools are employed for quantitative analysis.

Sphincter pharyngoplasty: Speech outcome and complications

Objectives: To assess the speech outcomes and complications in children who had undergone sphincter pharyngoplasty (SP) for management of velopharyngeal insufficiency (VPI). Study Design: Retrospective chart review. Methods: Charts from patients who had sphincter pharyngoplasty between January 1993 and June 1996 were reviewed. Syndrome diagnosis and presence of repaired cleft palate were reviewed. Preoperative speech assessment, videofluoroscopic and nasopharyngoscopic evaluations, age at time of surgery, and postoperative speech assessments were reviewed for all patients. Postoperative videofluoroscopy and nasopharyngoscopy were performed for those patients who had persistent VPI. Obstructive sleep symptoms were also assessed. Results: Thirty patients were identified; six patients had no follow-up evaluation, leaving 24 patients included in this study. Average follow-up was 11.7 months (range, 2–35 mo). Velocardiofacial syndrome (VCFS) was the most commonly identified syndrome. Postoperatively, 15 of 24 patients (62.5%) had complete resolution of their VPI; five of 24 (20.8%) had significant improvement; one of 24 (4.2%) had minimal to no change; and three of 24 (12.5%) were hyponasal. Of the six patients with some degree of persistent VPI, three underwent revision surgery. All three patients had complete resolution of their VPI after revision surgery, resulting in an overall success rate of 18 of 24 (75%). Conclusions: Sphincter pharyngoplasty has wide application in the management of children with VPI, including those with VCFS. The procedure is readily modified to accommodate an individual patient's needs as determined by preoperative VPI evaluation. A modification of the procedure is described to minimize the risk of postoperative airway obstruction and hyponasality, both regarded as airway complications of sphincter pharyngoplasty.     

Surgical planning for restoring velopharyngeal function in velocardiofacial syndrome

Background

Velocardiofacial syndrome (VCFS) is one of the most common multiple anomaly syndromes in humans. Around 70% of the cases show velopharyngeal insufficiency (VPI), as a consequence of cleft palate. VPI is much more frequent due to special abnormal conditions inherent to VCFS including: platybasia, hypotrophy of adenoid, enlarged tonsils, hypotonia and abnormal pharyngeal muscles.

Objective

To evaluate the surgical treatment of VPI in VCFS patients.

Materials and methods

In the Hospital Gea Gonzalez at Mexico City, all cases of VCFS from January 2000 to July December 2007 were studied. All patients subjected to velopharyngeal surgery for correcting VPI were selected. Twenty-nine patients underwent velopharyngeal surgery. All operations were planned according to findings of videonasopharyngoscopy (VNP) and multiview video fluoroscopy (MVF).

Results

Twenty patients underwent pharyngeal flap operations, and 9 patients were operated on with a sphincter pharyngoplasty. After a pharyngeal flap, 17 cases (85%) improved to normal nasal resonance or mild hypernasality. Three flaps showed moderate hypernasality postoperatively. From the 9 sphincter pharyngoplasties, 6 cases (66%) improved to moderate hypernasality. Four patients (33%) persisted with severe hypernasality postoperatively. There were no complications.

Conclusions

Tailor-made pharyngeal flaps seem to be the best option for restoring velopharyngeal function in cases of VPI in VCFS patients. The use of VNP and MVF is useful for planning the operations for VPI, and they are also useful for indicating the removal of tonsils in cases with high risk of obstruction. Moreover, VNP is also useful for preventing damage to the internal carotids which are usually displaced in VCFS patients.     

A speech nasoendoscopy-based surgeon’s decision for correction of velopharyngeal insufficiency following adenotonsillectomy

Both sphincter pharyngoplasty (SP) and pharyngeal flap (PF) procedures have gained popularity among surgeons as effective surgical management for velopharyngeal insufficiency (VPI). Different centers prefer either SP or PF and have published their results to support this preference. But is one technique superior to the other? To answer this question, we have adopted the concept of differential therapeutic management, based on detailed assessment of velopharyngeal function. According to this assessment either SP or PF was performed for management of VPI (secondary to adenotonsillectomy). The aim of this work is to evaluate and compare the surgical results after SP and PF (based on the preoperative nasoendoscopy and phoniatrics’ evaluation). This study was conducted on 31 patients with persistent hypernasality after adenotonsillectomy. All patients were subjected to perceptual speech evaluation and nasoendoscopic examination. According to this evaluation and data analysis, 18 patients were operated by SP and 13 patients underwent PF. Statistically, highly significant improvements were found when comparing pre- versus postoperative perceptual speech evaluation following both SP and PF (P < 0.001). When comparing the postoperative perceptual speech evaluation following both techniques, statistically non-significant differences were reported (P > 0.05). Preoperative differential diagnosis of VPI using perceptual speech assessment and nasoendoscopy of the velopharynx allows for tailored surgical management with either SP or PF. Both SP and PF procedures could yield good surgical outcomes, when patients are properly selected and the technique is chosen according to preoperative assessment.     

Partial adenoidectomy by suction diathermy in children with cleft palate, to avoid velopharyngeal insufficiency

Objectives

Adenoidectomy is indicated for the relief of paediatric nasal obstruction, sleep-disordered breathing and otitis media with effusion (OME). Velopharyngeal insufficiency (VPI) is a rare complication. The main risk factor is the presence of pre-existing velopharyngeal closure-impaired mechanisms, including submucosal or overt cleft palate. Despite possible benefits, adenoidectomy is frequently withheld in such children to avoid VPI. This study aims to demonstrate the efficacy and safety of partial adenoidectomy using suction diathermy in children who previously underwent overt cleft palate repair during infancy, to allow selective resection of tissue and symptom resolution without producing VPI.

Methods

Since 1994, 18 patients with previously corrected overt cleft palate have undergone partial adenoidectomy at this centre, for the treatment of nasal obstruction or sleep-disordered breathing, with or without OME. Three had existing VPI following their cleft correction surgery. Selective resection of the adenoid was performed transorally under indirect vision, using a malleable suction coagulator. This allowed exposure of the posterior choanae, leaving the remaining adenoid bulk intact.

Results

Patients were followed up at 4 weeks, and subsequently at regular intervals (total follow up 30-180 months, median 92 months), including perceptual speech assessment in all cases. All demonstrated symptomatic improvement with respect to the original indications for surgery. None developed worsening hypernasal speech or other features of VPI, and there were no cases of symptomatic adenoidal re-growth.

Conclusions

Partial adenoidectomy, employing a variety of methods, has been used successfully in children with submucosal cleft palate. This study demonstrates the safe and effective use of suction diathermy to enable partial adenoidectomy in children who have previously undergone surgical correction of overt cleft palate, allowing symptom resolution without producing VPI.     

Powered instrumentation for transnasal endoscopic partial adenoidectomy in children with submucosal cleft palate

Objectives

Partial adenoidectomy is the selective removal of the obstructing part of adenoid tissue, thus relieves obstruction symptoms and preserves the velopharyngeal valve action. Patients with palatal dysfunction are candidates for the technique. This study describes the use of microdebrider, transnasally (guided by the nasal endoscope) to perform partial adenoidectomy in patients with submucosal cleft palate, who presented with adenoidal hypertrophy and also it discusses its effects on nasal obstruction and speech.

Subjects and methods

This prospective study was carried out on twenty-three submucosal cleft palate patients who were referred to the ORL-HN department; Zagazig University Hospitals complaining of respiratory obstruction and sleep disturbances due to adenoids hypertrophy. After preoperative nasoendoscopic and speech evaluation, transnasal endoscopic, power-assisted partial adenoidectomy was done for all patients. All patients were followed up at regular visits including nasoendoscopy and speech evaluation.

Results

The procedure insured fast, safe, reliable, under vision and well controlled steps. Intraoperatively no major complications were recorded. During follow up, nasal obstruction and respiratory obstruction symptoms were improved. Speech outcome results were reported.

Conclusion

The study demonstrates the feasibility of using the microdebrider for performing transnasal partial adenoidectomy (under endoscopic guidance). The procedure is precise, rapid, safe and well-tolerated with the advantage of direct visualization of a traditionally difficult-to-expose area. The study reported improvement of respiratory obstruction symptoms with good speech results.     

Assessment of adenoid size: A comparison of lateral radiographic measurements, radiologist assessment, and nasal endoscopy

Objectives

Correlate adenoid size as determined by lateral neck radiographs and intra-operative mirror exam. Determine if a radiologist's assessment of the lateral neck X-ray correlates with adenoid size. Assess the correlation of endoscopic findings to the degree of adenoid hypertrophy seen on intra-operative mirror exam. To perform a cost analysis of radiographic and endoscopic evaluations of the adenoids.

Study design

Retrospective study.

Methods

Patients who underwent adenoidectomy were reviewed. The adenoid size as determined by the adenoid-to-nasopharyngeal (A/N) ratio, radiology report, and flexible nasal endoscopy were compared to the adenoid size as determined by intra-operative mirror nasopharyngeal exam. Compensation rates for each modality were compared.

Results

Sixty-one children had pre-operative airway radiography. Ninety-nine patients underwent flexible nasopharyngoscopy. When the A/N ratio was compared to the intra-operative mirror exam, the Pearson Correlation coefficient was 0.64 (p < 0.0001). The radiology reading was compared to intra-operative mirror exam and the Spearman Correlation coefficient was 0.29 (p = 0.0258). When endoscopic nasopharyngoscopy was compared to intra-operative mirror exam, the Pearson Correlation coefficient was 0.62 (p < 0.0001). The cost of nasal endoscopy was $654. Lateral airway radiography plus radiology interpretation cost $605.

Conclusion

Children who undergo lateral radiographs to assess adenoid size are younger than those who undergo awake flexible endoscopic nasopharyngoscopy. Both the A/N ratio and endoscopic nasopharyngoscopy correlate well with the findings of the intra-operative mirror exam. The radiologist interpretations that do not utilize the A/N ratio measurement do not correlate well with intra-operative mirror exam findings. Both modalities are comparable in cost.     

A middle ear implant with a titanium canal wall prosthesis for a case of an open mastoid cavity

Objective

Open mastoid cavity rehabilitation should focus on both anatomical and functional aspects. We hereby report the technique and results of a combined strategy to reconstruct the external ear canal using a titanium wall implant and the middle ear using a fully implantable active middle ear device.

Methods

A fully implantable active middle ear implant was used to rehabilitate the mixed hearing loss of a 63-year-old woman, and a titanium posterior canal wall prosthesis was used to reconstruct the external ear canal during the same procedure. The middle ear implant was placed directly on the footplate. The auditory results were compared to the preoperative unaided thresholds and to the amplification of a conventional hearing aid.

Results

Following the procedure, there was an anatomically normal external ear canal with a healed tympanic membrane separating the external from the middle ear spaces. The postoperative auditory gains were on average 31.8 dB on pure-tone audiometry, and 20 dB on speech reception threshold. No complications occurred.

Conclusion

The rehabilitation of the external ear canal in an open mastoid cavity allows for clinical follow-up of the patient, and the implantation of an active middle ear implant provides appropriate auditory gains both in pure tones and in speech reception thresholds.     

The role of the efferent auditory system in developmental dyslexia

Objective

To assess the role of the efferent auditory system by inhibition of contralateral otoacoustic emission in dyslexic children with auditory processing disorders.

Methods

The study sample was 34 children: 17 with dyslexia and 17 age-matched controls. Sensitive speech tests (low-pass filtered, time-compressed, distorted and dichotic) were performed to assess coexisting auditory processing disorder. Distortion-product otoacoustic emission (DPOAE) values were measured in basal condition and with contralateral broadband noise signal delivered via an earphone transducer at 60 dB SPL.

Results

The lower scores at sensitive speech testing confirmed the association of an auditory processing disorder in the dyslexic children. DPOAE values were significantly attenuated by contralateral inhibition only in the control group (p = 0.001; dyslexics, p = 0.19); attenuation was not significant at any frequency in the dyslexic group.

Conclusions

The differences in DPOAE attenuation between the groups, although not statistically significant, suggest alterations in the auditory efferent system in the dyslexic population. These alterations may affect language perception. If confirmed in further studies with larger samples, these results could provide insight into a possible pathophysiological background of dyslexia.     

Auditory performance and speech intelligibility of Mandarin-speaking children implanted before age 5

Objectives

(1) To report the auditory performance and speech intelligibility of 84 Mandarin-speaking prelingually deaf children after using cochlear implants (CIs) for one, two, three, four, and five years to understand how many years of implant use were needed for them to reach a plateau-level performance; (2) to investigate the relation between subjective rating scales and objective measurements (i.e., speech perception tests); (3) to understand the effect of age at implantation on auditory and speech development.

Methods

Eighty-four children with CIs participated in this study. Their auditory performance and speech intelligibility were rated using the Categorical Auditory Performance (CAP) and the Speech Intelligibility Rating (SIR) scales, respectively. The evaluations were made before implantation and six months, one, two, three, four, and five years after implantation. At the fifth year after implantation, monosyllabic-word, easy-sentence, and difficult-sentence perception tests were administered.

Results

The median CAP score reached a plateau at category 6 after three years of implant use. The median SIR arrived at the highest level after five years of use. With five years of CI experiences, 86% of the subjects understood conversation without lip-reading, and 58% were fully intelligible to all listeners. The three speech perception tests had a moderate-to-strong correlation with the CAP and SIR scores. The children implanted before the age of three years had significantly better CAP and monosyllabic word perception test scores.

Conclusions

Five years of follow-up are needed for assessing the post-implantation development of communication ability of prelingually deafened children. It is recommended that hearing-impaired children receive cochlear implantation at a younger age to acquire better auditory ability for developing language skills. Constant postoperative aural–verbal rehabilitation and speech and language therapy are most likely required for the patients to reach the highest level on the CAP and SIR scales.     

Speech outcomes at age 5 and 10 years in unilateral cleft lip and palate after one-stage palatal repair with minimal incision technique – A longitudinal perspective

Objectives

To investigate speech outcomes in 5- and 10-year-old children with unilateral cleft lip and palate (UCLP) treated according to minimal incision technique (MIT) – a one-stage palatal method.

Methods

A retrospective, longitudinal cohort study of a consecutive series of 69 patients born with UCLP, treated with MIT (mean age 13 months) was included. Forty-two children (43%) received a velopharyngeal flap; 12 before 5 years and another 18 before 10 years of age. Cleft speech variables were rated from standardized audio recordings at 5 and 10 years of age, independently by three experienced, external speech-language pathologists, blinded to the material. The prevalences of cleft speech characteristics were determined, and inter- and intra-rater agreement calculated.

Results

More than mild hypernasality, weak pressure consonants and perceived incompetent velopharyngeal function were present in 19–22% of the children at 5 years, but improved to less than 5% at 10 years. However, audible nasal air leakage, prevalent in 23% at 5 years, did not improve by age 10. Thirty percent had frequent or almost always persistent compensatory articulation at 5 years, and 6% at age 10. The general impression of speech improved markedly, from 57% giving a normal impression at 5 years to 89% at 10 years. A high prevalence of distorted/s/was found at both 5 and 10 years of age.

Conclusions

A high occurrence of speech deviances at 5 years of age after MIT was markedly reduced at 10 years in this study of children with unilateral cleft lip and palate. The high pharyngeal flap rate presumably accounted for the positive speech development.     

Results with sphincter pharyngoplasty and pharyngeal flap.

OBJECTIVE: To evaluate speech outcomes and complications of sphincter pharyngoplasty and pharyngeal flap performed for management of velopharyngeal insufficiency (VPI). DESIGN: Case series. SETTING: Tertiary care children's hospital. PATIENTS: All patients who underwent pharyngeal flap or sphincter pharyngoplasty from 1990 to 1995. METHODS: Perceptual speech analysis was used to assess severity of VPI, presence of nasal air emissions and quality of nasal resonance (hyper, hypo, or normal). Pre-operative measures of velopharyngeal function were based upon nasendoscopy and videofluoroscopic speech assessment. Recommendations for management were made by the attending surgeon. Complications of hyponasality and obstructive sleep symptoms (OSS) were noted. Patient characteristics were compared using univariate analysis. RESULTS: Sixteen patients underwent sphincter pharyngoplasty and 18 patients underwent superiorly based pharyngeal flap. Patients were similar in terms of lateral pharyngeal wall medial motion and palatal elevation. The groups were also similar with regard to VPI severity, though there was a trend for more severe VPI in patients undergoing sphincter pharyngoplasty than pharyngeal flap (50 vs. 33.3%, respectively). Patients with pharyngoplasty had a higher rate of resolution of VPI than those who had pharyngeal flap (50 vs. 22.2%, respectively), although this was not statistically significant. Post-operative hyponasality and obstructive sleep symptoms were present in both groups. However, only patients who underwent PF and had postoperative OSS had obstructive sleep apnea (OSA). CONCLUSIONS: There were no detectable anatomic differences between treatment groups implying that treatment selection during the study period was not guided by strict anatomic criteria. Sphincter pharyngoplasty may have a higher success rate with a lower risk of OSS.     

The role of the medial olivocochlear system in the complaints of understanding speech in noisy environments by individuals with normal hearing

Objective

The aim of our study is to investigate the relationship between the complaint of speech understanding in noisy environments and the findings of contralateral suppression of transient evoked otoacoustic emissions and speech recognition in noise test methods in individuals with normal hearing.

Methods

Sixty-nine subjects between 18 and 53 years of age with normal hearing participated in the present study. The subjects were assigned to one of two groups, reported difficulty understanding speech in noise or no reported difficulty understanding speech in noise. After hearing and immitancemetric evaluation, contralateral suppression of transient evoked otoacoustic emissions and speech recognition in noise tests were administered to both groups. Suppression was calculated in half-octave frequency bands centered at 1.0, 1.5, 2.0, 3.0 and 4.0 kHz.

Results

We found out that the speech recognition in noise scores and contralateral suppression values were lower in subjects with the complaint of speech understanding in noise than those who do not have such complaints.

Conclusions

We concluded that the complaint of speech understanding in noise may be related to the medial efferent system dysfunction, so central auditory nervous system.     

Endoscopic partial medial maxillectomy with mucosal flap for maxillary sinus mucoceles

Purpose

To describe a technique of endoscopic medial maxillectomy with mucosal flap for postoperative maxillary sinus mucoceles and to present a case series of subjects who underwent this procedure.

Materials and methods

This case series includes four subjects with postoperative maxillary sinus mucoceles who underwent resection via endoscopic partial medial maxillectomy with a mucosal flap. We will discuss the clinical presentation, imaging characteristics, operative details, and outcomes.

Results

Four subjects are included in this study. The average age at the time of medial maxillectomy was 52 years (range 35–65 years). Three subjects (75%) were female. One subject (25%) had bilateral postoperative maxillary sinus mucoceles. Two subjects (50%) had unilateral right sided mucoceles, and the remaining subject had a unilateral left sided mucocele.All subjects had a history of multiple sinus procedures for chronic sinusitis including Caldwell–Luc procedures ipsilateral to the postoperative mucocele. All subjects underwent endoscopic medial maxillectomy without complication and were symptom free at the last follow up appointment, average 24 months (range 3–71 months) after medial maxillectomy.

Conclusions

For postoperative maxillary sinus mucoceles in locations that are difficult to access via the middle meatus antrostomy, we recommend endoscopic medial maxillectomy with mucosal flap. Our preliminary experience with four subjects demonstrates complete resolution of symptoms after this procedure.     

The occurrence of velopharyngeal insufficiency in Pierre Robin Sequence patients

Objective

Children born with Pierre-Robin Sequence (PRS) have cleft palate, micrognathia, and macroglossia. After the repair of the cleft palate, velopharyngeal insufficiency (VPI) can occur in a subset of patients. We hypothesize that the need for the surgical correction of VPI in PRS children is no different than cleft palate only (CPO) patients.

Methods

A retrospective study of 21 children with non-syndromic PRS who were matched to 42 non-syndromic, CPO controls for age and sex. We reviewed incidence of VPI, the need for secondary speech surgery, and speech outcomes post-operatively.

Results

Secondary surgery to correct VPI was necessary in 3 of 21 (14.29%) PRS patients (2 Pharyngeal Flaps, 1 Z-plasty), vs. 10 of 42 (23.81%) CPO (9 Pharyngeal Flaps, 1 Z-plasty) controls. Mean age for VPI surgery for PRS vs. controls: 5.33 vs. 6.41 years, respectively. For final speech outcomes, 73.68% of PRS vs. 71.88% of controls showed no evidence of hypernasality, 89.47% of PRS patients vs. 93.75% of controls showed no evidence of hyponasality, and 76.47% of PRS patients vs. 78.13% of controls had normal velopharyngeal competence (p > 0.90 for all three measures).

Conclusion

Our findings suggest that children born with a Pierre-Robin Sequence do not have a higher rate of post-operative VPI after cleft palate repair and are no more likely to require additional surgical intervention.     

Simultaneous translabyrinthine removal of acoustic neuroma and cochlear implantation

Objectives

To report of a 65-year-old woman with bilateral Meniere's disease was referred for cochlear implantation (CI) due to severe/profound sensorineural hearing loss.

Methods

During the assessment workup, a vestibular schwannoma in the right ear was found by MR imaging. She underwent a translabyrinthine removal of the acoustic neuroma (AN) with sparing of the cochlear nerve and concurrent ipsilateral CI with a Nucleus Freedom device (Cochlear Ltd., Lane Cove, New South Wales, Australia).

Results

Complete removal of the AN was achieved without complications. Neural Response Telemetry (NRT) measurements, which showed poor morphology at the intraoperative tests, rapidly improved after activation, similarly to electrically evoked auditory brainstem responses (E-ABR). The patient reached 100% speech perception performances within 2 months from implantation, in the monaural condition. She was relieved from vertigo spell up to 14 months after the operation.

Conclusion

Cochlear implantation at the time of acoustic neuroma removal with VIII nerve sparing can be a safe and effective hearing restoration procedure.     

Keratinic amyloidosis of the external auditory canal

Objective

We present a rare case of keratinic amyloidosis of the external auditory canal. This is only the seventh case reported of localized cutaneous amyloidosis of the external auditory canal with no systemic symptoms.

Patient

A 62-year-old man, who had complained of an itchy external auditory canal and left-side hearing loss, was referred to our hospital because of a bilateral external auditory canal mass.

Intervention

Biopsy of the external auditory canal mass suggested a diagnosis of amyloidosis. However, total systemic examination failed to identify any disease due to systemic amyloidosis. This led us to diagnose him with localized cutaneous amyloidosis of the external auditory canal.

Main outcome measure

We follow up periodically with systemic examination and local observation.

Results

Thirty months after the initial diagnosis, he remains in follow-up and has not shown any significant aggravation of the disease.

Conclusion

In previous cases, the chief complaints were itching sensations and pain in the external auditory canal as well as a sense of discomfort when wearing a hearing aid. This suggests that chronic stimulation and inflammation of the skin lining the external auditory canal induced amyloidosis.     

Comparing the effect of auditory-only and auditory–visual modes in two groups of Persian children using cochlear implants: A randomized clinical trial

Objective

Since the introduction of cochlear implantation, researchers have considered children's communication and educational success before and after implantation. Therefore, the present study aimed to compare auditory, speech, and language development scores following one-sided cochlear implantation between two groups of prelingual deaf children educated through either auditory-only (unisensory) or auditory–visual (bisensory) modes.

Design

A randomized controlled trial with a single-factor experimental design was used.

Methods

The study was conducted in the Instruction and Rehabilitation Private Centre of Hearing Impaired Children and their Family, called Soroosh in Shiraz, Iran. We assessed 30 Persian deaf children for eligibility and 22 children qualified to enter the study. They were aged between 27 and 66 months old and had been implanted between the ages of 15 and 63 months. The sample of 22 children was randomly assigned to two groups: auditory-only mode and auditory–visual mode; 11 participants in each group were analyzed. In both groups, the development of auditory perception, receptive language, expressive language, speech, and speech intelligibility was assessed pre- and post-intervention by means of instruments which were validated and standardized in the Persian population.

Results

No significant differences were found between the two groups. The children with cochlear implants who had been instructed using either the auditory-only or auditory–visual modes acquired auditory, receptive language, expressive language, and speech skills at the same rate.

Conclusion

Overall, spoken language significantly developed in both the unisensory group and the bisensory group. Thus, both the auditory-only mode and the auditory–visual mode were effective. Therefore, it is not essential to limit access to the visual modality and to rely solely on the auditory modality when instructing hearing, language, and speech in children with cochlear implants who are exposed to spoken language both at home and at school when communicating with their parents and educators prior to and after implantation.The trial has been registered at IRCT.ir, number IRCT201109267637N1.