Congenital external auditory canal stenosis and partial atretic plate

Objectives

To report outcomes of surgery for severe congenital external auditory canal (EAC) stenosis with or without partial atretic plate (PAP).

Design

Retrospective review.

Subjects

Thirteen patients (18 ears) had surgery for severe EAC stenosis with (n = 10, 56%) or without PAP (n = 8, 44%). Indications included severe stenosis with hearing loss, cerumen impactions, and/or canal cholesteatoma. Mean age = 7.8 years (range 0.4-19.9 years). Mean follow up = 5.2 years (range 0.4-10.0 years).

Setting

Tertiary care children's hospital.

Interventions

Nineteen endaural canaloplasties were performed in 17 ears (2 revisions). There was one post-auricular approach. Fifteen tympanoplasties were performed in 13 ears (2 revisions). PAP was reconstructed with drilling to enlarge the bony annulus and fascia grafting to enlarge the tympanic membrane in 10 (56%) ears.

Outcome measures

Patency of EACs; otologic findings; pure tone averages (PTA); complications.

Results

All (100%) EACs had improved patency (≥4 mm). Findings included canal cholesteatoma (2/18, 11%), ossicular fixation (4/18, 22%), stapes abnormality (2/18, 11%), and incudostapedial discontinuity from cholesteatoma (1/18, 6%). Mean preoperative PTA = 38.7 dB HL (range 60-20 dB HL). Mean post-operative PTA = 23.6 dB HL (range 50-6.7 dB HL). Audiologic results were significantly better for cases without PAP (p < .01) and without ossicular fixation (p < .01). There were seven minor and no major complications.

Conclusions

Endaural canaloplasty is safe and effective for providing patent EACs and hearing improvement for severe congenital EAC stenosis. However, since hearing outcomes were worse for cases with PAP and ossicular fixation, alternatives such as hearing aids or BAHA^® may be considered.     

Characteristics of hearing impairment in Yemeni children with chronic suppurative otitis media: A case-control study

Background

Chronic suppurative otitis media (CSOM) is a serious disorder particularly in low resource settings. It can lead to disabling hearing impairment and sometimes life-threatening infective complications.

Objective

The aim of the present study was to describe the characteristics of hearing impairment associated with CSOM in Yemeni children.

Methods

A case-control study of 75 children with CSOM and 74 healthy controls. Hearing was assessed by behavioural testing and audiometry.

Results

Cases had lower academic performance than controls (OR 15.31, 95% CI 1.99-322.14, p < 0.001). Disabling hearing impairment >30 dB was present in 51.5% (right ear) and 66.7% (left ear) of children with CSOM.

Conclusion

Disabling hearing impairment was identified as a major health problem in these Yemeni children with CSOM. There is a need for investment to reduce the burden of CSOM and its complications in these communities. Greater attention to the chronic disabling effects of CSOM in children is required in poor communities and low resource settings.     

Congenital incudostapedial anomalies in adult stapes surgery: a case-series review

Objectives

The management of developmental anomalies of the ossicular chain and otic capsule in stapes surgery tests the otologist's diagnostic skills and flexibility in technique and prosthesis choice. We review our experience in managing various incudostapedial developmental anomalies discovered during middle ear exploration for suspected otosclerosis in adult patients.

Design

A retrospective case-series review was used.

Setting

The study was set at a military tertiary referral center.

Patients and other Participants

A review of 185 stapes procedures yielded 8 cases of identified congenital anomalies of the stapes or incudostapedial complex including congenital absence of the oval window with malformed incus and stapes, columellar-type stapes with promontory fixation, stapes superstructure fixation to the fallopian canal, and malformed incus with stapes superstructure fixation to the promontory.

Interventions

The developmental anatomy and surgical technique in each case were reviewed. The surgical technique used in each case varied depending on the specific ossicular abnormality found and whether both the stapes and incus were affected.

Main outcome measures

Comparison of preoperative and postoperative audiometric testing results was performed.

Results

All 8 ears (7 patients) that underwent a stapes procedure for either an isolated congenital stapes anomaly or a combined incudostapedial anomaly had a favorable hearing outcome, with 7 ears improving to within 10-dB and 1 ear to within 13-dB air-bone gap on postoperative audiometry.

Conclusions

Despite unanticipated findings of congenital anomalies of the stapes and incus during middle ear exploration for conductive hearing loss, a flexible management approach can lead to successful hearing outcomes.     

Treatment of persistent middle ear effusion in cleft palate patients

Objectives

The goals of the research project are to learn how to individualize otologic care for cleft palate patients and to be able to counsel families of children with cleft palate on the benefit of tympanostomy tubes, hearing issues and risks of multiple sets of tubes.

Methods

The study is a retrospective chart review. Patients with a cleft palate with or without a cleft lip born between 1 January 2000 and 31 December 2005 referred to the Connecticut Children's Medical Center Craniofacial Department were included in the study. The patients were offered individualized ear surgery (PE tube placement) only if persistent middle ear fluid was present for over 3 months with a conductive hearing impairment. The primary outcome measures included the newborn hearing screening results, number of ear tube surgeries, and complications of PE tube insertion.

Results

There were 86 patients with cleft palate spectrum with or without cleft lip (45 females and 41 males). Twelve had undocumented newborn hearing evaluations. Of the 74 evaluable results, 61 (82%) passed the newborn hearing screening, 8 (11%) failed and 5 (7%) were inconclusive. By 5 years old, 84 (98%) patients received at least one set of ear tubes for persistent middle ear fluid with conductive hearing impairment, while 2 received no tubes (2%). Of those who received ear tubes, the range was 1-6 with a mean of 1.7. Twelve patients (14%) had tympanosclerosis. Eight patients (9%) had eardrum perforation. One patient had myringoincudopexy. Of the 86 patients, 12 had undocumented newborn hearing evaluations. Of the 74 evaluable results, 61 (82%) passed the newborn hearing screening, 8 (11%) failed and 5 (7%) were inconclusive.

Conclusions

(1) The majority of children born with cleft palate do not have middle ear fluid at birth. (2) Most children with cleft palate will likely develop persistent middle ear fluid with conductive hearing loss. Risks of complications from ear tubes in cleft palate patients are few and manageable using standard sized ear tubes.     

Choice of approach for revision surgery in cases with recurring chronic otitis media with cholesteatoma after the canal wall up procedure

Objectives

Cholesteatoma has a tendency to recur if not properly eradicated. This study sought to investigate and compare the outcome of the canal wall up (CWU) versus the canal wall down (CWD) procedure for recurrent cholesteatoma after initial canal preserving surgery.

Methods

Between January 1990 and August 2007, 42 patients who underwent a revision tympanomastoidectomy for a recurred cholesteatoma were analyzed retrospectively. All patients initially underwent the canal wall up procedure. Recurrence rates, audiologic outcomes, and the extent of recurrent/residual cholesteatoma were investigated, and the revision surgical methods were compared.

Results

The mean follow-up duration was 10 years (range, 13 months-15.6 years). The CWD procedure was performed in 29 (69%) patients with a recurred cholesteatoma and the CWU procedure in 13 (31%) as a first revision procedure. CWD surgeries were performed in more severe cases. A second revision surgery was required in five (12%) patients. Extended cholesteatoma recurrences were observed even among cases with a lower disease stage at the time of primary surgery. The second recurrence rate was significantly higher in the CWU group than the CWD group (p = 0.026). The 8-year disease-free follow-up rate in the CWD group was significantly higher than the CWU group (p = 0.002). Postoperative AB gap closure was significantly better in the CWU group than CWD group (p = 0.001).

Conclusion

The CWD procedure is a safer and more successful method for controlling recurrent cholesteatoma. Thus, surgeons should not be hesitant to perform the CWD procedures for revision cases.     

Hearing results of stapedotomy and malleo-vestibulopexy in congenital hearing loss

Aims

To analyze hearing results of surgical treatment of hearing loss associated with the congenital stapes ankylosis with or without malformations of ossicular chain.

Study design

Retrospective chart review.

Methods

The charts of 1369 stapedotomies performed by senior author (JH) from 1991 to 2006 were reviewed. In 40 cases operative findings were consistent with isolated congenital stapes fixation or associated with middle ear malformations. The modified stapedotomy technique was employed in 33 cases and malleo-vestibulopexy was used in 7 cases. Operative findings were standardized according to Cremers’ classification. The outcomes of 40 surgeries were analyzed according to the 1995 AAO-HNS Committee on Hearing and Equilibrium guidelines. High frequency hearing results on 4, 8 and 12 kHz were reported in addition to standard frequencies. Results of stapedotomies and malleo-vestibulopexies were calculated separately. Surgical complications were described.

Results

The mean post-operative air conduction (AC) was 33 dB, bone conduction (BC) 22 dB and speech reception thresholds (SRT) 31 dB. Closure of the air-bone gap (ABG) to within 10 dB was achieved in 24/40 (60%) of cases. Lack of improvement was observed in 3/40 (8%) patients. In 26/32 (81%) of cases with potential for bilaterally serviceable hearing it was achieved. In 24/40 (60%) of cases symmetrical hearing with interaural difference of less than 10 dB was demonstrated.

Conclusion

Significant hearing gain in patients with congenital stapes ankylosis makes surgical treatment a valuable adjunct or an alternative to hearing aids in selected cases.     

Bone-anchored hearing aids (Baha) in congenital aural atresia: personal experience

Aims

Aural atresia is a congenital disease constituted by partial or complete lack of development of the external auditory canal, which is generally associated with malformations of the auricle and middle ear.Reconstruction of the auditory canal and correction of any deformities of the middle ear have yielded unpredictable results and variable functional outcomes, and there is a high rate of complications. Therefore, the use of bone-conduction hearing aids, such as the Baha, may represent a valid alternative for subjects who have conductive hearing loss with cochlear reserve that, as a rule, is fully conserved.The aim of this work is to reexamine our experience with the management of conductive and mixed hearing loss using the Baha system in children with bilateral aural atresia.

Methods

We examined 31 patients with bilateral congenital aural atresia in whom a Baha system had been implanted. The patients, 16 males and 15 females, were between 5 and 14 years of age (mean 8.7).The following parameters were assessed for each patient: mean preoperative air and bone conduction for frequencies between 0.5 and 4 kHz; mean preoperative threshold with conventional bone-conduction hearing aids; mean postoperative threshold with the Baha system; improvement in quality of life evaluated with the Glasgow Children's Benefit Inventory; rate and type of surgical complications.

Results

The mean preoperative air- and bone-conduction thresholds were 51.2 ± 12.5 and 14.1 ± 6.3 dB HL, respectively. The mean preoperative threshold with a conventional bone-conduction hearing aid was 29.3 ± 7.2 dB HL, and the mean postoperative threshold with the Baha system was 18.1 ± 7.5 dB HL. Quality of life improved for all operated patients.

Conclusions

The results of our study of the Baha system to treat patients with bilateral aural atresia were extremely satisfactory compared both with those of surgical reconstruction of the auditory canal and those of traditional bone-conduction hearing aids. Furthermore, great improvement was noted in quality of life, while the rate of complications was very low.Therefore, we are convinced that the Baha system is the treatment of choice for hearing loss due to bilateral congenital aural atresia.     

Acquired medial external auditory canal stenosis,anterior tympanomeatal angle blunting,and lateralized tympanic membrane: Nosology,diagnosis, and treatment

Objectives

To analyze the etiologies, auditory consequences, diagnostic tools and therapeutic results of three often confused pathologies: acquired fibrous stenosis of the medial part of the external auditory canal (EAC), fibrous anterior tympanomeatal angle blunting, and lateralized tympanic membrane.

Epidemiological analysis of Microtia: A retrospective study in 345 patients in China

Objective

To study the epidemiologic characteristics of microtia in China and to investigate the possible risk factors with respect to the classification of microtia.

Methods

A total of 345 patients with microtia were studied. All patients were taken an intentional physical examination and classified into five types. A detailed questionnaire concerning the maternal conditions during pregnancy was filled out by patient's mother. The frequencies of the relative factors were counted and the variables were statistically analyzed using Chi Square and Fisher's tests in the five types of microtia.

Results

Most cases of microtia (88.12%) were sporadic and 72.75% of all cases occurred in males. It was more seen unilateral, especially affected on the right side (55.94%). A total of 195 patients (56.52%) were isolated microtia. In the rest of non-isolated microtia patients, 37.97% of the cases had hemifacial microsomia, which was the most common associated deformity. Thirty-four patients (9.86%) belonged to typical familial microtia. Three maternal factors showed significant differences in the five types of microtia, which were perinatal virus infection, high prior miscarriages and prevention treatments for threatened abortion.

Conclusions

The majority of microtia cases in China are sporadic and usually more common in males. Mothers who have prior miscarriages over 3 times or perinatal virus infection seem to be more likely to have severe microtia infants.     

Prevalence and etiology of congenital or early acquired hearing impairment in Eastern Finland

Objective

The purpose of this study was to determine the prevalence and etiology of congenital or early acquired bilateral sensorineural hearing impairment (SNHI) in children born from 1988 to 2002 in the district of Kuopio University Hospital, Finland, and to compare the results with those from an earlier 14-year period in the same region and similar population.

Methods

The data were collected retrospectively from Hospital Records. The degree of hearing impairment was based on average air conduction threshold calculated over the frequencies 0.5, 1, 2 and 4 kHz in the better hearing ear. Hearing impairment was classified as mild (≥20-39 dB), moderate (40-69 dB), severe (70-95 dB) and profound (>95 dB).

Results

We identified 92 children with bilateral SNHI diagnosed before the age of 7 years. The overall prevalence and the prevalence for at least moderate SNHI was 2.1 per 1000 live births and 1.2 per 1000 live births, respectively. We found no differences in the prevalence of SNHI during both study periods. Etiology was genetic in 46%, acquired in 14% and unknown in 40%. Out of the genetic cases 74% were non-syndromic and 26% were syndromic. In comparison to the previous study there was a decline in the proportion of acquired SNHI and the proportion of genetic and unknown cause had increased. Six children in five families had homozygous 35delG mutation and six children in four families presented with a homozygous M34T mutation.

Conclusions

The prevalence of congenital or early acquired SNHI in the Kuopio University Hospital district area has not changed during a period of 29 years. Despite possibility to test the GBJ2 gene, the proportion of hearing impairment of unknown etiology remained high.     

The audiological characteristics of a hereditary Y-linked hearing loss in a Chinese ethnic Tujia pedigree

Objective

To investigate audiometric characteristics of hearing loss in a large Chinese ethnic Tujia family and determine its hereditary type.

Methods

Total 76 live individuals were investigated in the notable 84 members of this family. The detailed audiometric evaluations were undertaken for the proband and his 47 family members. The degrees of sensorineural hearing impairment were defined as an air/bone gap <15 dB hearing loss averaged over 0.5, 1 and 2 kHz. The severity of hearing loss was established based on the hearing ability of the better ear, averaged over 0.5, 1, 2 and 4 kHz, and classified into four categories: mild, moderate, severe and profound.

Results

Nineteen patrilineal relatives of the 76 live members had hearing impairment. The age of onset ranged from 7 to 21 years old with the average of 13.2 years. The audiometric defect was described by auditory curves of a high frequency in 47% of the patients. Affected members in this family demonstrated a non-syndromic, late onset, bilateral, symmetrical, postlingual and sensorineural hearing loss.

Conclusions

The audiometric configuration in males of the pedigree is consistent with the hereditary Y-linked hearing loss. Thus we speculate that a putative gene on the Y chromosome could contribute to the cause of the disease.     

Non-organic hearing loss in childhood

Objective

The present study aimed to investigate the etiology, symptoms, diagnosis and prognosis of pediatric patients with non-organic hearing loss (NOHL), and to heighten awareness of this disorder among physicians.

Methods

Between January 2000 and July 2009, we retrospectively reviewed the medical records of 47 pediatric patients (aged 6-18 years of age) diagnosed with NOHL. The diagnosis was made when there were audiometric discrepancies between the subjective and objective hearing thresholds of the patient in the absence of any organic disease.

Results

Eighteen patients presented with unilateral hearing loss, and 29 showed bilateral hearing loss. Five patients received steroid treatment before the correct diagnosis was made, and six had secretory otitis media and underwent a tympanostomy tube placement.

Conclusion

If physicians are unaware of the possibility of NOHL; they may misdiagnose children with idiopathic sudden sensorineural hearing loss and administer high-dose steroid treatments or exploratory tympanotomies. Otoacoustic emissions are abolished when NOHL patients have secretory otitis media. In these cases, after tympanostomy tube placement, they should undergo objective electrophysiologic examinations to reevaluate NOHL.     

A comparison of the proliferative capacity and ultrastructure of proliferative cells from the cochleae of newborn rats of different ages

Objective

Recent reports have shown that multipotent stem cells/progenitor cells that are capable of proliferation and regeneration are present in mammalian cochleae. However, progenitor cells have not been isolated from the adult cochlea. We examined the proliferative potential of cells derived from neonatal rats of various ages. The determination of the differences between the proliferative cells from rats of different ages may provide clues to the mechanisms controlling the destiny of these cells.

Methods

Proliferative cells were isolated from the cochleae of 1-, 7-, and 14-day-old rats, and the proliferative capacity and ultrastructure of the cells from each age group were assessed using flow cytometry and transmission electron microscopy, respectively.

Results

During the first two postnatal weeks, the number of proliferative cells gradually fell to zero. This decrease occurred in parallel with the impairment of the proliferative capacity of the cells and the accumulation of proliferative cells in G0/G1. In addition, some of the cells exited the cell cycle by means of gradual maturity and apoptosis.

Conclusions

Our study suggests that cochlear proliferative cells are remnants of the progenitor cells that originally gave rise to the sensory epithelium. The disappearance of the cochlear proliferative cells in adult mammalian cochleae may result from their differentiation and/or apoptosis.     

Colchicine prolongs patency of myringotomy in an animal model

Objective

Chronic otitis media with effusion (COME) is a frequently observed condition in childhood. The most common and effective surgical therapy for COME is myringotomy with insertion of a ventilation tube (VT). Our aims were to investigate the combined effect of myringotomy and the topical application of Colchicine solution to the external ear canal for the prolongation of patency in the treatment of patients with COME and to evaluate the ototoxicity of Colchicine applied directly to the middle ear.

Methods

A prospective study on 47 ears in 26 fat sand rats was fashioned. In the first phase, solutions of different concentrations of Colchicine were applied to the middle ear cavity in order to determine the drug's ototoxicity, assessing inner ear function with ABR. In the second phase myringotomy was performed and a non ototoxic concentration of Colchicine applied to 12 external ear canals, while saline was applied to nine.

Results

In the first phase, Colchicine concentration of 0.1% and higher applied to the middle ear cavity caused an ABR threshold elevation. In the second phase, the mean closure time after 0.01% Colchicine application was prolonged to >2.14 weeks (P < 0.05).

Conclusion

Colchicine has a proven potential for prolongation of myringotomy patency when applied as a solution to the external ear as a 0.01% solution. Further investigations are required to validate these results in humans and to study the potential effect of repetitive Colchicine application on the duration of myringotomy patency for the treatment of COME.     

Suction cautery adenoidectomy (SCA): is the additional cost justified?

Objectives

Adenoidectomy is one of the oldest and most frequent ENT procedures. This study aimed to compare adenoidectomy using suction-cautery adenoidectomy (SCA) to curettage with respect to operative time, postoperative complications, and cost-effectiveness.

Methods

The data for this retrospective case control study were retrieved from the Medical Records Department at one of the few medical centers that perform this technique in the Kingdom of Saudi Arabia. The data for each case included the following: patient demographic features, type of procedure, time of operation, occurrence of any postoperative complications, length of hospital stay and cost of the procedure. To minimize the sources of variance in our data, all adenoidectomies were performed by the same consultant otolaryngologist, using either SCA or curettage.

Result

Of the 86 patients who underwent adenoidectomy in this study, SCA was performed in half of them (43) and curettage in the other half. The two groups were well matched with no significant group differences in either age or gender (p = 0.2 and p = 0.19, respectively). There was a significant reduction in operative time (p < 0.001) in the SCA group. There were no cases of postoperative hemorrhage after SCA, but there was one case of hemorrhage in the curette group that required a 2nd surgery to control the bleeding. Regarding cost, there were additional profits of more than 700,000 SR (US$180,000) each month with SCA as compared to curettage.

Conclusions

The suction cautery technique was superior at reducing operative time, increasing cost-effectiveness and decreasing the risk of postoperative complications. Therefore, we suggest suction cautery as the most appropriate method for adenoidectomy.     

Isolated malleus-handle fracture

Objective

Isolated malleus fracture is a rare ossicular injury with less than 80 cases published in the literature. The aim of this paper is to present a series of 3 patients with isolated malleus handle fracture, to review the literature and to discuss clinical presentation, imaging findings and treatment options.

Methods

We retrospectively a case series of 3 patients with isolated malleus handle fracture managed at a tertiary referral otologic center.

Results

The three patients related the same history of digitally manipulating the external auditory canal, leading to a sudden hearing loss and feeling of aural fullness. Meticulous otomicroscopy revealed the broken malleus with excessive movement of the distal part of the malleus on pneumatic otoscopy. High-resolution CT scan with multiplanar reconstruction along the axis of the malleus confirmed the fractures and displacements. One patient was treated conservatively and two underwent ossiculoplasty.

Conclusion

Malleus handle fracture should be systematically suspected in case of hearing loss and aural fullness of sudden onset after digital manipulation of the external auditory canal.     

A comparison of audiometric and objective methods in hearing screening of school children. A preliminary study

Objective

In newborn hearing screening, one exclusively applies objective hearing testing methods - based on evoked potentials and/or on otoacoustic emissions. However, when testing school children, one can consider both audiometric and electrophysiological methods. The choice of methods is determined by the aims of the program. If one wants to detect conductive hearing losses, impedance audiometry seems to be the method of choice.

Methods

The aim of this study was to compare test performance measures from audiometric and objective methods (OAEs and impedance audiometry), in the hearing screening of school children. Screening protocols were applied on a group of 190 children of about 12 years of age (6th grade of primary school).

Results

For a single application of a screening procedure, the best performance was observed in the automated four-tone audiometry, followed by the tympanometry and the TEOAE-based procedures. Screening performance was enhanced using a combination of automated and impedance audiometry. A four-tone audiometry test combined with tympanometry gives a sensitivity of 65%, and the PPV of 46%, which are reasonable values, acceptable for practical use. The use of a TEOAE protocol degrades the overall performance of screening.

Conclusions

Screening of school children is feasible with a combination of automated audiometry and tympanometry with time requirements equal to 3 min per subject.     

Measuring communicative performance with the FAPCI instrument: preliminary results from normal hearing and cochlear implanted children

Objective

: To develop preliminary “growth curves” of Functioning after Pediatric Cochlear Implantation (FAPCI) scores using a cross-sectional sample of normal hearing children and to compare these curves to trajectories of FAPCI scores in children receiving cochlear implants.

Methods

: Quantile regression was used to develop growth curves from the FAPCI scores of a cross-sectional sample of 82 normal hearing children (age range 7 months-5 years). Trajectories of FAPCI scores from a longitudinal cohort of 75 children with cochlear implants (age range 1-5 years) were compared to these growth curves.

Results

: FAPCI scores were positively associated with increasing age in normal hearing children with a rapid increase in scores observed at earlier ages followed by a plateau at age 3 years. FAPCI trajectories for cochlear-implanted children varied with age at implantation and did not reach a plateau until age 5-6 years.

Conclusion

: Normal hearing children demonstrated increasing FAPCI scores with age, and these preliminary growth curves allow for the interpretation of a cochlear-implanted child's FAPCI scores in comparison to normal hearing children. Additional research using a larger, longitudinal cohort of normal hearing children will be needed to develop definitive normative FAPCI trajectories.     

The development of auditory skills in infants with isolated Large Vestibular Aqueduct Syndrome after cochlear implantation

Objective

The purpose of this study was to investigate the auditory performance of infants with isolated Large Vestibular Aqueduct Syndrome (LVAS) after cochlear implantation, compare their performance with those of infants with a normal inner ear, and establish a database of auditory development.

Method

435 infants with congenital severe to profound hearing loss participated in this study. 62 infants in group A were diagnosed with isolated LVAS. 373 infants in group B had a normal inner ear. Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) was used to evaluate the development of auditory skills.

Results

The mean scores for auditory ability showed no significant difference between groups A and B. The mean scores for the three different auditory skills increased significantly over time. The differences were statistically significant in mean scores among the three different auditory skills for group B.

Conclusion

Auditory skills of infants with isolated LVAS developed rapidly after cochlear implantation, in a similar manner to those of infants with a normal inner ear. Cochlear implantation is an effective interventional approach and an established therapeutic option for infants with isolated LVAS.