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.     

Bilateral secondary neurolymphomatosis of the internal auditory canal nerves: A case report

Background

Neurolymphomatosis describes the malignant lymphomatous infiltration of nerves.

Methods

We encountered a unique case of a 47-year-old patient with non-Hodgkin's lymphoma presenting with bilateral sensorineural hearing loss, vestibular dysfunction and bilateral facial nerve palsy.

Results

Magnetic resonance imaging demonstrated enhancement and thickening of internal auditory canal nerves bilaterally consistent with neurolymphomatosis. Patient was treated with combined intrathecal chemotherapy and total brain irradiation.

Conclusions

One must always remain vigilant for metastatic disease in patients with sensorineural hearing loss and/or vestibular dysfunction and facial nerve palsy in the context of known malignancy.     

Sudden hearing loss with simultaneous posterior semicircular canal BPPV: Possible etiology and clinical implications

Objectives

The objectives of this study were to describe the clinical course and outcome of patients with sudden sensorineural hearing loss (SSNHL) in conjunction with benign paroxysmal positional vertigo (BPPV), and hypothesize the possible pathophysiology of this entity.

Study design

Retrospective study of all patients with evidence of SSNHL with any type of BPPV between 2008 and 2012.

Settings

Tertiary care university hospital.

Subjects and methods

Five patients aged 56 to 71 were diagnosed with unilateral profound SSNHL and BPPV. Neurotologic examination revealed an ipsilateral torsional, up-beating nystagmus on Dix–Hallpike exam. Severe or profound ipsilateral–sensorineural hearing loss was recognized on audiometry. The rest of the exam was normal; this was in keeping with the diagnosis of SSNHL with ipsilateral posterior semicircular canal BPPV.

Results

All patients were treated with a modified Epley maneuver; oral steroids were administered for two weeks. In all cases vertigo resolved and the Dix–Hallpike exam became normal within several weeks. However, the hearing loss remained unchanged in two patients. Magnetic resonance imaging of the head was normal and ENG caloric test demonstrated mild ipsilateral canal paresis in two patients.

Conclusions

1. Patients with SSNHL and BPPV can have a variable clinical course and outcome. This entity may be quite common, but the diagnosis of BPPV can be missed if a complete neurological physical examination is not performed. 2. Arterial occlusions or selective multiple vascular or neural involvement may explain the pathophysiology of SSNHL with BPPV of the posterior semicircular canal.     

The effectiveness of the promotion of newborn hearing screening in Taiwan

Objective

Hearing is a critical ability for the development of a child's speech and language. Many studies in different countries have shown the universal newborn hearing screening and early intervention has greatly reduced the negative impact caused by congenital hearing loss. The first universal newborn hearing screening program in Taiwan took place in MacKay Memorial Hospital in 1998 and was subsequently endorsed by the government. The incidence of bilateral congenital hearing impairment in Taiwan is approximately 2.6 per 1000 live birth. The aim of this paper is to analyze the age of diagnosis, hearing aid fitting, and intervention of congenitally hearing impaired children with and without hearing screening after public awareness and government endorsement of newborn hearing screening.

Materials and methods

There were 263 hearing impaired children participated in this study, receiving their auditory habilitation therapy at Children's Hearing Foundation from 2006 to 2010. 114 of those children went through newborn hearing screening and 149 without it. The age of diagnosis, hearing aid fitting, and auditory intervention were compared between these two groups. The age of diagnosis and intervention of congenitally hearing impaired children among different years were analyzed too.

Results

The average age of diagnosis was 8.7 months, the age of hearing aid fitting was 12.4 months and age of auditory intervention was 18.8 months for the group of hearing impaired children with newborn hearing screening. For hearing impaired children without newborn screening, their average age of diagnosis was 27.5 months; age of hearing aid fitting was 31.3 months and age of auditory intervention was 40.5 months. There were significant differences in the age of diagnosis, hearing aid fitting and auditory intervention between congenitally hearing impaired children with and without hearing screening.

Conclusions

This research indicates that newborn hearing screening facilitates early identification, diagnosis and intervention of congenitally hearing impaired children in Taiwan. The age of identification, diagnosis and intervention of congenital hearing impaired children has also been reduced gradually over the years after government endorsement of newborn hearing screening in Taiwan.     

Mastoid obliteration using calcium phosphate bone paste with an artificial dermis soaked with basic fibroblast growth factor: preliminary clinical report

Objectives

To describe mastoid obliteration using a calcium phosphate paste with an artificial dermis soaked with basic fibroblast growth factor (b-FGF) and the usefulness of this procedure.

Methods

Ten ears that underwent a canal wall-down mastoidectomy were treated with calcium phosphate paste. The cases consisted of four patients with mastoid cavity problem, four with middle ear cholesteatoma, one with adhesive otitis media, and one with external auditory canal cholesteatoma. The post-operative follow-up period was 19.9 months on average (ranging from 13 to 23 months). First, the canal wall-down mastoidectomy was performed. Then, the calcium phosphate paste was used to fill up to the facial ridge posteriorly and the horizontal segment of the facial canal superiorly. The surface of the paste was covered with an artificial dermis soaked with b-FGF.

Results

Following calcium phosphate paste treatment, all open cavities were decreased in volume and epithelized within 2 months.

Conclusion

Calcium phosphate paste appears effective for mastoid obliteration. Using an artificial dermis soaked with b-FGF appears to prevent exposure of the paste to the skin of the external auditory meatus. However, the number of the patients is too small to draw any conclusion.     

Evidence for genotype–phenotype correlation for OTOF mutations

Objectives

The aim of this study is to evaluate the auditory phenotype in subjects with OTOF gene mutations to describe genotype–phenotype correlations.

Methods

Twenty-two affected members from three families with homozygous OTOF mutations were included. Nine subjects were evaluated audiologically with otoscopic examination, pure-tone audiometry, tympanometry with acoustic reflex testing, auditory brain stem responses, and otoacoustic emission tests.

Results

Homozygous c.4718T>C (p.Ile1573Thr) mutation was associated with the auditory neuropathy/auditory dys-synchrony (AN/AD) phenotype and with progressive sensorineural hearing loss in four siblings in one family, while homozygous c.4467dupC (p.I1490HfsX19) was associated with severe to profound sensorineural hearing loss without AN/AD in four relatives in another family. Homozygous c.1958delC (p.Pro653LeufsX13) mutation was associated with moderate sensorineural hearing loss without AN/AD in one affected person in an additional family.

Conclusions

The audiological phenotype associated with different OTOF mutations appears to be consistently different suggesting the presence of a genotype–phenotype correlation.     

External auditory canal stenting utilizing a useful rolled, tapered silastic sheet (RTSS) post middle ear surgery

Objective

To overcome the demerits of conventional postoperative aural packing, we developed a useful protocol for postoperative stenting of the external auditory canal after middle ear surgery which enables transcanal drainage and simultaneously allows for visual inspection and treatment of the canal, as necessary.

Methods

Twenty-four surgeries, 21 patients underwent tympanoplasty with a postaural incision. At the end of all surgical procedures, the external auditory canal was packed with a 0.3 mm thickness rolled, tapered silastic sheet (RTSS) with antibiotic ointment applied to one surface. The inserted RTSSs were removed at 5–10 days postoperatively. We assessed the efficacy and the reliability of the RTSS.

Results

In 23 ear surgeries on 20 patients, we achieved successful postoperative ear packing utilizing our RTSS. With these patients, the tympanic membrane and the external auditory meatus were able to be observed immediately after the completion of the stenting during the surgery and the removal of the gauze over the operated ear at Day 1 to Day 3 postoperatively. During 4 surgeries with ventilation tube insertion to the tympanic membrane, there was secretion through the inserted ventilation tube which was easy to suction. In one surgery, on one patient, additional packing materials were utilized once only during a sandwich graft myringoplasty. No patients showed any harmful effects during the postoperative period.

Conclusions

The useful and reliable RTSS, with antibiotic ointment applied to one surface, has several positive advantages that the conventional packing methods do not.     

Hearing loss in vestibular schwannomas: analysis of cochlear function by means of distortion-product otoacoustic emissions

Objective

We investigated cochlear function in a group of patients affected by vestibular schwannoma (VS), by means of recording distortion-product otoacoustic emissions (DPOAEs).

Methods

Between January 1996 and January 2007, we observed 183 patients affected by unilateral VS. DPOAEs, compared to the corresponding hearing thresholds, were subjectively classified into three categories: “compatible” with hearing function, “cochlear” and “retro-cochlear”. We also related the responses to some clinical variables (tumor size, intracanalicular tumor and radiologic appearance of the internal auditory canal). Statistical analysis was performed.

Results

In 137 cases (74.9%), DPOAEs were as expected based on audiometry responses, while in 11 patients (6%) a “cochlear” DP-gram was recorded and in 35 patients (19.1%) DPOAEs evidenced a “retro-cochlear” pattern. In eight cases we detected acoustic responses despite a profound hearing loss. No statistically significant data merged from the comparison between “cochlear” and “retro-cochlear” responses and the clinical variables.

Conclusion

Our results confirm that sensorineural hearing loss due to VS can be of sensory and/or neural origin. DPOAEs still remain just a complementary auditory test; nevertheless, in case of severe or profound unilateral hearing loss, recorded acoustic responses may be suspicious for the presence of a vestibular schwannoma.     

Cochlear implantation in patients with chronic otitis media

Objective

Patients with complications of otitis media present a significant challenge to safe cochlear implantation. We describe our experience of cochlear implantation in patients with chronic ear disease, and propose management principles according to the presenting status of the ear.

Methods

Cochlear implantations were performed as treatment for complications of otitis media in seven patients. They consisted of four patients with adhesive otitis media, two patients with an open cavity after surgery for otitis media and one patient with eosinophilic otitis media.The electrodes were inserted by an approach via the external auditory canal in patients with poor growth of the mastoid antrum or adhesion of the tympanum. For the patients with an open cavity, we created a posterior wall for the external auditory canal and perform the mastoid obliteration. Modified Rambo's technique with blind sac closure of the external auditory canal was performed for the case of eosinophilic otitis media as a single-stage procedure.

Results

The post-operative courses were good. However, a post-operative infection developed in one patient who had previously undergone radiation therapy following surgical excision of a cerebellar tumor.

Conclusion

Transcanal approach is effective in a poorly pneumatized mastoid or severe adhesive otitis media.A decision whether implantation as a single-stage or multiple stages depends on the condition of each cases. But there is a possibility of infection even if we selected a stage operation.     

Hearing threshold assessment in young children with electrocochleograpy (EcochG) and auditory brainstem responses (ABR): Experience at the University Hospital of Ferrara

Objective

Electrophysiological evaluation is a fundamental procedure for the diagnostic assessment of hearing loss during infancy; in these cases, information concerning threshold level and auditory perception is particularly useful to establish a correct hearing rehabilitation program (hearing aids and cochlear implants).Purpose of this study is to underline the role of auditory brainstem responses (ABR) and electrocochleography (EcochG) in the definition of hearing loss in a selected group of children, referred to the Audiology Department of the University Hospital of Ferrara, for a tertiary level audiological assessment.

Methods

A retrospective study of the paediatric patient database at the Audiology Department of the University Hospital of Ferrara has been performed. In a period between January 2000 and December 2007, a total of 272 paediatric cases have been identified (544 ears).An EM 12 Mercury apparatus has been used for the electrophysiological threshold identification (ABR and EcochG). Recordings were carried out under general anaesthesia, in a protected enviroment.

Results

In 19 of the 272 paediatric cases selected—38 ears (7%), the results of threshold evaluation through ABR were uncertain. The Ecochg recording resulted crucial for the final diagnosis in terms of definition of the hearing threshold level, and it was then possible to ensure the better hearing rehabilitation strategy.

Conclusions

ABR has to be considered the first choice in hearing assessment strategy, either for screening or for diagnosis in newborns as well as in non-collaborating children; ECochG still may be considered a reliable diagnostic tool.     

External auditory canal microbiology and hearing aid use

Objective

The aim of this study was to determine the presence and nature of bacterial flora on hearing aids and the ears of this population. We wonder if the microbiology of the ears with hearing aid wearing differs from the other ear.

Setting

Tertiary referral center.

Design

A prospective, clinical study.

Subjects and methods

Three samples were taken, one from the surface of the hearing aid's ear mold; one from the hearing aid-wearing ear canal and the last one from the ear without hearing aid. Samples were cultured to determine qualitatively and quantitatively the pathogenic microorganisms present.

Results

A total of 123 samples, obtained from 41 hearing aid users, were analyzed. Methicillin-resistant coagulase-negative staphylococci, methicillin-susceptible Staphylococcus aureus, methicillin-resistant S. aureus, Pseudomonas aeruginosa, Escherichia coli, Acinetobacter species, Staphylococcus auricularis, and Stenotrophomonas maltophilia were identified organisms.

Conclusions

We identified unexpected microorganisms both on hearing aids and hearing aid using ears. This study demonstrates that using hearing aid alters the ear canal flora. To avoid otitis externa, it is important to use an appropriate hygiene routine to clean and disinfect hearing aids and ear molds.     

Auditory processing disorders: Relationship to cognitive processes and underlying auditory neural integrity

Background

Auditory processing disorder (APD) in children has been reported and discussed in the clinical and research literature for many years yet there remains poor agreement on diagnostic criteria, the relationship between APD and cognitive skills, and the importance of assessing underlying neural integrity.

Purpose

The present study used a repeated measures design to examine the relationship between a clinical APD diagnosis achieved with behavioral tests used in many clinics, cognitive abilities measured with standardized tests of intelligence, academic achievement, language, phonology, memory and attention and measures of auditory neural integrity as measured with acoustic reflex thresholds and auditory brainstem responses.

Method

Participants were 63 children, 7–17 years of age, who reported listening difficulties in spite of normal hearing thresholds. Parents/guardians completed surveys about the child's auditory and attention behavior while children completed an audiologic examination that included 5 behavioral tests of auditory processing ability. Standardized tests that examined intelligence, academic achievement, language, phonology, memory and attention, and objective tests auditory function included crossed and uncrossed acoustic reflex thresholds and auditory brainstem responses (ABR) were also administered to each child.

Results

Forty of the children received an APD diagnosis based on the 5 behavioral tests and 23 did not. The groups of children performed similarly on intelligence measures but the children with an APD diagnosis tended to perform more poorly on other cognitive measures. Auditory brainstem responses and acoustic reflex thresholds were often abnormal in both groups of children.

Summary

Results of this study suggest that a purely behavioral test battery may be insufficient to accurately identify all children with auditory processing disorders. Physiologic test measures, including acoustic reflex and auditory brainstem response tests, are important indicators of auditory function and may be the only indication of a problem. The results also suggest that performance on behavioral APD tests may be strongly influenced by the child's language levels.     

Landmarks for the preservation of the middle temporal artery during mastoid surgery: Cadaveric dissection study

Importance

The middle temporal artery flap is a vascularized periosteal flap that is highly useful for otologic reconstruction including the middle cranial fossa, mastoidectomy defect, and external auditory canal. The course of the artery is close to the external auditory canal and is easily injured during preliminary exposure and elevation of flaps.

Autism and peripheral hearing loss: A systematic review

Objective

To systematically review the literature describing the relationship between autism spectrum disorder (ASD) and peripheral hearing loss including literature recommendations for audiological assessment and auditory habilitation in cases where peripheral hearing loss and ASD coexist.

Data sources

Published studies indexed in MEDLINE (1948–2011).

Review methods

The search strategy identified 595 potential studies. After a review of the titles, 115 abstracts were reviewed and 39 articles were retrieved and assessed independently by at least two authors for possible inclusion. 22 articles pertained to children with ASD and peripheral hearing loss, hearing assessment in children with ASD, audiological habilitation for children with ASD or hyper-responsiveness in children with ASD. 17 further studies were garnered from the reference section of the 22 papers.

Results

Controversy exists in the literature regarding prevalence of hearing impairment among individuals with ASD. In cases where ASD and hearing impairment co-exist, diagnosis of one condition often leads to a delay in diagnosing the other. Audiological assessment can be difficult in children with ASD and test–retest reliability of behavioural thresholds can be poor. In cases where hearing impairment exists and hearing aids or cochlear implantation are recommended, devices are often fit with special considerations for the child with ASD. Hyper-responsiveness to auditory stimuli may be displayed by individuals with ASD. Evidence or the suspicion of hyper-responsiveness may be taken into consideration when fitting amplification and planning behavioural intervention.

Conclusions

Prevalence rates of hearing impairment among individuals with ASD continue to be debated. At present there is no conclusive evidence that children with ASD are at increased risk of peripheral hearing loss. A complete audiological assessment is recommended in all cases where ASD is suspected so as not to delay the diagnosis of hearing impairment in the event that hearing loss and ASD co-exist. Objective assessment measures should be used to confirm behavioural testing in order to ensure reliability of audiological test results. Fitting of hearing aids or cochlear implantation are not contraindicated when hearing loss is present in children with ASD; however, success with these devices can be variable.     

Evaluation of cortical processing of language by use of positron emission tomography in hearing loss children with congenital cytomegalovirus infection

Objective

To predict cochlear implant efficacy and investigate the cortical processing of the visual component of language in profoundly deafened patients with asymptomatic congenital cytomegalovirus (CMV) infection.

Methods and cases

The cortical activity of two children with CMV-related hearing loss was evaluated with fluorodeoxyglucose-positron emission tomography (FDG-PET) with a visual language task before cochlear implantation. Total development and auditory perception ability were assessed one year after implantation.

Results

The two children with CMV-related hearing loss showed activation in the auditory association area where no activation was found in the controls, and exhibited nearly identical cortical activation patterns to those seen in patients with profound congenital hearing loss. In contrast, differences in total development in verbal ability and discrimination of sentences between the two cases were revealed one year after implantation.

Conclusion

These results might indicate that the differences of cortical activities according to hearing abilities could have been influenced by CMV infection that involves higher function of the brain directly and/or affects the cochlea peripherally. Additionally, if CMV infection might have affected only the cochlea, these cortical activation patterns were influenced secondary by the time course of hearing loss characterized by CMV infection, which had varied manifestations.Accurate diagnosis and cochlear implantation at the appropriate time are important for successful speech development, and each patient needs a personalized habilitation program based on their etiology and brain function.     

Quantification of hearing loss associated with superior semi-circular canal dehiscence

Introduction

Superior semi-circular canal dehiscence (SSCD) is a known cause of hearing loss. This study quantifies hearing loss in SSCD ears in a frequency-specific fashion.

Methods

A meta-analysis of English language literature pertaining to SSCD was performed, with extraction and evaluation of available human audiometric data. Our own institution's case series of SSCD patients was also similarly analysed. Hearing loss in SSCD ears was compared to same patient control ears and to age-matched normative audiometric data.

Results

Ears with SSCD had statistically significant worse hearing as compared to both normative data and to own normal ear controls at 2000 Hz and below. The effect appears to diminish with increasing frequency.

Discussion

The presence of statistically significant conductive hearing loss in the low frequencies was confirmed for SSCD ears. SSCD may also predispose ears to high frequency sensorineural hearing loss.     

Primary paraganglioma in the facial nerve canal

Objective

To describe primary paraganglioma in the facial nerve canal and discuss the characteristics of facial nerve paraganglioma in contrast with other tumors.

Case report

A 23-year-old man developed gradually progressive right facial palsy as the initial symptom. One year later, he exhibited hearing loss without tinnitus in his right ear. CT demonstrated an enlarged facial nerve canal with irregular bony erosion of the circumference. MRI showed a well-enhanced heterogeneous mass with hypo-intensity spots inside it. During surgery, a blood-rich tumor was observed along the facial nerve: however, extensive bleeding interfered with tumor removal. The surgical specimen demonstrated paraganglioma. The tumor was completely removed in the second surgery in combination with arterial embolization. Facial nerve function was reconstructed with a free muscle flap more than one year following resection.

Conclusion

Because paraganglioma is a blood-rich tumor, it is important to perform angiography and embolization. If preoperative facial nerve palsy is demonstrated, careful management of facial nerve function is needed. Paraganglioma must be considered in the differential diagnosis of a facial nerve tumor.     

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.     

Radiological quiz. Sudden sensorineural hearing loss due to multiple sclerosis

Objective

A case with sudden sensorineural hearing loss (SSNHL) owing to multiple sclerosis (MS) who had clinical and dramatic radiological improvement just after medical therapy was reported in this article.

Method

Case report and review of related literature.

Results

A 22-year-old female patient with MS related SSNHL was presented in this article. Magnetic resonance imaging (MRI) revealed an MS plaque localized at pons extending from right cochlear nucleus to proximal part of the right cochlear nerve. Most dramatic recovery was present in the 5th day control MRI, where the plaque located on pons disappeared completely. On the 10th day control audiogram hearing recovery was observed and pure tone audiogram levels were almost normal.

Conclusion

Sudden sensorineural hearing loss owing to MS is seen more common than expected. It has good prognosis. Magnetic resonance imaging is also thought to have an important role in diagnosis and treatment efficacy of SSNHL owing to MS.     

Malignant paroxysmal positional vertigo

Objective

An insidious percentage of paroxysmal positional vertigo appears to be intractable with canalith repositioning maneuver and also is not self-limiting. This type of positional vertigo is sustained by the action of intracranial tumors that mimics the clinical aspects of benign paroxysmal positional vertigo.Aim of this study is to clarify the features of these forms of positional vertigo, which we indicate as malignant paroxysmal positional vertigo.

Methods

We retrospectively reviewed the clinical records of all the patients who presented with vertigo spells and were managed at our tertiary care referral centre over a three years period. Two hundred and eleven patients with diagnosis of positional paroxysmal vertigo were included in the final study.

Results

Seven patients were affected by intracranial tumors causing a positional vertigo and were classified as malignant paroxysmal positional vertigo patients after radiological and histological diagnosis. These patients were affected by an internal auditory canal mass alone or with extension in the cerebello pontine angle that mimicked a benign positional vertigo.

Conclusion

We can conclude that the clinician should keep in mind the differentiation between benign positional vertigo and malignant positional vertigo. When the patients with positional vertigo presents a strange behaviour of symptoms, nystagmus or response to the canalith repositioning maneuver a radiological investigation must be undertaken in every doubtful case.