Bilateral Hearing Research Articles

Large Vestibular Aqueduct Syndrome: A Case Study

A 23-month-old female was referred for hearing aid fitting after failing newborn hearing screening and being diagnosed with significant hearing loss through subsequent diagnostic testing. Auditory brainstem response (ABR) and behavioral testing revealed a moderate-to-severe bilateral mixed hearing loss. Prior to the hearing aid evaluation, tympanostomy tubes had been placed bilaterally with little or no apparent change in hearing sensitivity. Initial testing during the hearing aid fitting confirmed earlier findings, but abnormal middle ear results were observed, requiring referral for additional otologic management. Following medical clearance, binaural digital programmable hearing aids were fit using Desired Sensation Level parameters. Behavioral testing and probe microphone measures showed significant improvements in audibility. Decrease in hearing sensitivity was observed six months following hearing aid fitting. Radiological studies, ordered due to the mixed component and decreased hearing sensitivity, revealed large vestibular aqueduct syndrome (LVAS). Based on the diagnosis of LVAS, a cochlear implant was placed on the right ear; almost immediate speech-language gains were observed.

Unilateral versus bilateral bone-anchored hearing aids (BAHAs)

Unilateral versus bilateral bone-anchored hearing aids (BAHAs)

Unilateral versus bilateral bone-anchored hearing aids (BAHAs)

Unilateral versus bilateral bone-anchored hearing aids (BAHAs)

Long-term clinical course and temporal bone histology after cochlear implantation

Objectives Illustrate long-term follow-up clinical and histologic data from a patient with unilateral multichannel cochlear implantation.Design Case report.Methods Clinical history, evolution of audiometric findings, and results of temporal bone histopathology of a patient implanted with a Nucleus 22 system in the right ear were analysed and interpreted.Results Audiometry showed severe bilateral sensorineural hearing loss, which progressed in the non-implanted ear postoperatively. With the implanted ear, speech perception was excellent postoperatively and remained stable over 5 years. Histology showed a relatively small number of spiral ganglion cells bilaterally.Conclusions Cochlear implantation can provide stable hearing in light of deteriorating hearing loss in the non-implanted ear. Only a small number of surviving ganglion cells were necessary for the implant.

Antiphospholipid Inner Ear Syndrome

This study was initiated to clarify the possible association between antiphospholipid antibodies, mediators of microthrombus formation, and sensorineural hearing loss (SNHL) of unknown origin in a large cohort of patients. Prospective study. The study cohort consists of 168 adult patients (>/=18 yr) referred to the University of Pennsylvania's Division of Neurotology and Balance Center for diagnosis and treatment of progressive hearing loss with or without vertigo. A comprehensive screening panel of blood tests for autoimmune and infectious diseases was applied to all patients, including testing for anticardiolipin antibodies, anti-B2 glycoprotein, and lupus anticoagulant. Forty-two patients (25%) had at least one elevated antiphospholipid antibody marker. Twenty patients had two or more positive test results. Of the 42 patients, 64% (n = 27) met the diagnostic criteria for Meniere disease, and the remainder were diagnosed with idiopathic SNHL. Within this group of patients, 24 patients (57%) had unilateral hearing loss, and 18 (44%) had bilateral hearing loss. These data support the hypothesis that antiphospholipid antibodies are involved in the pathogenesis of some forms of inner ear dysfunction, presumably by causing microthrombus formation in the labyrinthine vasculature. Basic science studies are required to better understand the mechanisms by which antiphospholipid antibodies mediate inner ear dysfunction. Clinical studies to evaluate the efficacy of anticoagulation in this group of patients are also required.

Meningeal carcinomatosis causing isolated bilateral symmetric progressive hearing loss

A 53-year-old man presented with isolated bilateral progressive hearing loss from about 5 months. Fourteen months after onset of this isolated symptom a diagnosis of meningeal carcinomatosis (MC) was made. Isolated bilateral deafness is a rare first manifestation of MC.

顕微鏡的多発血管炎に伴った変動する感音難聴例

Microscopic polyangitis (MPA) is one of the systemic vasculitis syndromes, including polyarteritis-nodosa and allergic granulomatosis angitis, which are characterized by clinical evidence of blood vessel inflammation that produces ischemic changes in various organs. Here we report a patient who showed bilateral fluctuating sensorineural hearing loss and diagnosed as having microscopic polyangitis. A 71-year-old woman visited our hospital complaining of redness on the nasal tip and alas. One week later she noticed tinnitus and hearing loss in the right ear, and then hearing of both ears fluctuated and responded well to the administration of predonisolone. She was suspected of having an autoimmune disease because she complained of numbness of fingers and erythema of the face. By close examination, she was diagnosed as having microscopic polyangitis and was given predonisolone and cyclophosphamide. Her bilateral hearing has been stable and MPO-ANCA became negative after the therapy. The sensorineural hearing loss in MPA may be caused by necrotic or inflammatory vasculitis in the inner ear. We should pay attention to the association of an autoimmune disease such as MPA in cases of fluctuating or repeated sensorineural hearing loss responding well to the administration of steroids.

Cochlear implantation in Waardenburg's syndrome

Conclusions. Children with Waardenburg's syndrome (WS) derive significant benefit from cochlear implantation (CI) and do so to an extent that is comparable to that of the general population of implanted children. Although we report on a relatively small cohort, our data are useful for counseling the parents of children with WS considering CI. Objective. To present our experience with CI in patients with WS. Material and methods. A retrospective record review was conducted for five children who underwent CI in our department between 1993 and 2004. Results. Children with WS comprised 1.9% of our entire pediatric CI population: four girls had a familial history of WS and the phenotype of WS Type I, and one boy met the criteria for WS Type II. They were all diagnosed as having bilateral profound sensorineural hearing loss 4–24 weeks after birth. Rehabilitation was initiated immediately and included bilateral fitting of hearing aids and intensive speech and language therapy. Otoscopic and temporal bone high-resolution CT findings were normal in all patients. At surgery, all children were found to have a patent cochlea, and the electrodes were implanted into the scala tympani without difficulty. After 1.3–10.2 years of implant use all children achieved open-set recognition of 2-syllable words, with an average score of 81%. Four of the 5 children achieved open-set recognition of monosyllabic words (average score 40%) and 3 achieved open-set recognition of words in sentences (average score 81%).

Ｔｒｅａｃｈｅｒ Ｃｏｌｌｉｎｓ症候群例

Patients with Treacher Collins syndrome often exhibit conductive hearing loss due to middle ear malformation. We report a case of this syndrome in a 41-year-old woman with bilateral conductive hearing loss, in whom adjustment of hearing devices was complicated. After assessing HRCT images using Jahrsdoerfer's grading scale, we performed tympanoplasty type III-c on the left side followed by type III-i on the right side. The patient had an excellent outcome; ultimately hearing devices were not required. Preoperatively, patients with congenital anomalies of the middle ear can benefit greatly from prediction of postoperative prognosis; use of Jahrsdoerfer's grading system to assess HRCT images can help to ensure safe and effective surgical care.

Paget’s disease and cochlear implantation

Paget's disease of bone is a common disorder of unresolved etiology characterized by excessive bone resorption followed by excessive bone formation. If the skull is affected this may result in hearing loss and eventually develop into profound deafness. To date, no cases of cochlear implantation in patients with Paget's disease have been reported. The authors present a case of radiographically confirmed Paget's disease of the skull in a 77-year-old man with a 20-year history of progressive bilateral sensorineural hearing loss who underwent cochlear implantation. A successful insertion of the Nucleus 24 Contour electrode array was achieved without surgical and postoperative complications. At the 10 months' postoperative evaluation, the patient had gained useful open-set speech perception. In quiet conditions, his performance scores on the word and sentence recognition tests were 100 and 98 per cent, respectively. In the presence of noise (at +10 dB. signal-to-noise ratio), his performance scores on the word and sentence recognition tests were 96 and 94 per cent, respectively.

Otitis media and hearing loss among 12-16–year-old Inuit of Inukjuak, Quebec, Canada

Objectives. Chronic otitis media (COM) and associated hearing loss is a frequent problem for many Inuit children in Canada. In this study, we evaluated individuals aged 12–16 years living in Inukjuak, to determine the prevalence of middle ear disease and hearing loss, and the effect of hearing loss on academic performance. Methods. Otological examination, hearing test, medical and school file review were performed in November 1997. 88 individuals were seen. Results. Otological examination revealed maximal scarring in 1.8%, minimal scarring in 34.9%, normal eardrums in 49.1% and chronic otitis media in 16.9%. There were 62 individuals whose ear exams could be directly compared with a previous exam done in 1987. Of those, there were three ears that had developed COM and 4/13ears with COM in 1987 that had healed. Hearing tests found bilateral normal hearing in 80% (PTA <20dB), unilateral loss in 15% and bilateral loss in 5%. Hearing loss was associated with poorer academic performance in Language (p<.05). A similar trend was found in Mathematics but not in Inuttitut. Conclusion. Chronic otitis media remains a significant problem among the Inuit, with a prevalence of 16.9% in individuals aged 12-16 years. One in five in this age group has hearing loss, and this hearing loss impacts on academic performance.

Cochlear implantation in a post-irradiated patient for nasopharyngeal carcinoma

This patient is a 47-year-old man with nasopharyngeal carcinoma and had received radiotherapy six years previously. Bilateral progressive hearing loss was noted after radiotherapy, reaching total hearing loss one year ago. Hearing aids became useless to him. After a series of routine evaluations for cochlear implantation, as well as promontory test which showed positive on the left, cochlear implantation was conducted by the senior author (TSH) on 1 July 2003. During surgery, severe fibrotic granulation in the mastoid and middle ear was found, which was removed and cleaned. In the mean time, the incus was to be removed to gain access to the middle ear for cochleostomy and an entire active electrode array was inserted into the cochlea, using Nucleus 24 Contour. To prevent postoperative infection, intraoperative topical antibiotics (gentamicin) soaked in tempolaris muscle strips were loosely packed in the middle ear and mastoid. The postoperative course was uneventful without wound infection.

Hearing impairment in familial X-linked hypophosphatemic rickets.

Hearing impairment in patients with X-linked dominant hypophosphatemic rickets (XLH) is likely part of the natural history of the disease, developing during adulthood. Therefore, whereas close follow up and hearing monitoring is recommended in adults, serial audiograms in children with XLH are not justified. Furthermore, in a child with XLH and hearing impairment, other etiologies should be explored. Hearing impairment in XLH patients has been reported a few times in the 1970s and 1980s [1, 2, 4, 5]; however, it was our impression that our pediatric XLH patients did not manifest clinical hearing impairment. Therefore, in order to better clarify the association between XLH and hearing status, and consequently provide information regarding patients’ management, we prospectively evaluated the hearing status in our XLH patients and their afflicted parents. Hearing evaluations including audiometry, tympanometry and stapedial reflex thresholds recording were performed in 16 children with XLH (age 1–18 years, median 9.0 years, six males) and all of their ten afflicted parents (age 22–55 years, three male). None had a history of treatment with ototoxic drugs, noise exposure, previous ear surgery or chronic ear disease. Of 16 children, 15 demonstrated normal hearing. One with bilateral profound hearing loss was found to have a Mondini congenital inner ear malformation, documented by a temporal bone CT scan. Two adult males and one female, all with severe XLH-related orthopedic manifestations, demonstrated sensorineural hearing loss. More than two decades passed since hearing impairment was first described in XLH [5]. Table 1 summarizes the main findings of previous studies that have reported on this association, showing evidence of hearing impairment in a significant percentage of adult XLH patients versus minimal incidence of hearing loss in children [1, 2, 4, 5]. We compared the hearing status in a relatively large group of XLH children in a wide range of ages with that of their afflicted parents. None of our children had a hearing impairment attributable to XLH. The only child with hearing impairment was the one with a Mondini inner ear malformation which is believed to result from a developmental arrest of the membranous labyrinth during the first trimester of pregnancy [3], and is not known to be associated with rickets which is a post-natal disease. In contrast to the normal hearingwe found in ourXLH children, three of their ten parents had sensorineural hearing loss. These patientswere under 52 years old; hence presbyacusis was ruled out. Since in all three etiologies other than the disease process were excluded and in the light of the fact that all had severe orthopedic manifestations of rickets, we suspect that their hearing impairment is part of the disease process. They were referred to adult otolaryngologists for further management. Even though two of the earlier studies [2, 5] found hearing impairment in a very few children, later studies which investigated much larger cohorts of children [1, 4] as well as ours, did not find evidence of XLH-related hearing impairment in childhood. Therefore, it cannot G. Fishman Department of Pediatric Otolaryngology, ‘‘Dana’’ Children’s Hospital, Tel-Aviv Medical Center, ‘‘Sackler’’ School of Medicine, Tel-Aviv University, Tel-Aviv, Israel

Bilateral Cochlear Implants in Adults and Children

To measure the benefit (ie, sound localization and speech intelligibility in noise) of bilateral cochlear implants (CIs) in adults and in children. Seventeen adults and 3 children underwent testing 3 months after activation of bilateral hearing. Adults received their devices in a simultaneous procedure and children in sequential procedures (3-8 years apart). Adults underwent testing of sound localization and speech intelligibility, with a single CI and bilaterally. Children underwent testing of sound localization, right/left discrimination, and speech intelligibility, with the first CI alone and bilaterally. We used computer games to attract the children's attention and engage them in the psychophysical tasks for long periods of time. Preliminary findings suggest that, for adults, bilateral hearing leads to better performance on the localization task, and on the speech task when the noise is near the poorer of the 2 ears. In children, localization and discrimination are slightly better under bilateral conditions, but not remarkably so. On the speech tasks, 1 child did not benefit from bilateral hearing. Two children showed consistent improvement with bilateral hearing when the noise was near the side that underwent implantation first. Bilateral CIs may offer advantages to some listeners. The tasks described in this study might offer a powerful tool for measuring such advantages, especially in young children. The extent of the advantage, however, is difficult to ascertain after 3 months of bilateral listening experience, and might require a more prolonged period of adjustment and learning. Future work should be aimed at examining these issues.

Is deafness a disease of poverty? The association between socio-economic deprivation and congenital hearing impairment

An association between congenital hearing impairmentand deprivation has been suggested, but evidence is limited.We studied children born in Greater Glasgow,1985-94, with bilateral congenital hearing impairment.The children were divided into seven deprivation categoriesusing the Carstairs Deprivation Index. One hundredand twenty-four hearing-impaired children wereborn over the study period, an incidence of 1.18/1000 livebirths. There was a clear association between deprivationcategory and incidence, ranging from 0.47/1000 to1.72/1000. An association with deprivation was seen forchildren with a family history and perinatal problems(such as prematurity and low birth weight). No associationwas found for other aetiological groups such ascraniofacial syndromes or early postnatal infection.Deprivation had no effect on age of diagnosis or hearingaid provision. Deprivation is associated with congenitalhearing impairment, due to more prematurity and lowbirth weight in deprived families, and the fact that familieswith many hearing-impaired members are economicallydisadvantaged. There should be a strong emphasison the needs of the socio-economically disadvantagedwhen planning services for hearing-impaired children.

Improving aetiological investigation of permanent hearing impairment in children

This paper describes the improvement in the aetiological investigation of children with permanent hearing impairment in one complete audit cycle over a four-year period. An aetiological investigation worksheet was produced and inserted into case notes of children with newly diagnosed bilateral permanent hearing loss with >40 dBHL in the better hearing ear over 0.5-4 kHz. There was a marked increase in the use of appropriate investigations and a significant increase in parental hearing tests and ECGs in those notes with and without the aetiology worksheet in the 2004 audit. There was also a significant increase in ophthalmology referral in cases with the worksheet. The aetiological investigation worksheet appears to be a useful tool for arranging appropriate investigation for the hearing impaired child.

Cochlear implant in Jervell and Lange‐Nielsen syndrome

The Jervell and Lange‐Nielsen syndrome is one of the best known and most extensively studied deafness syndromes inherited as an autosomal recessive trait. Its hallmarks are congenital profound bilateral sensorineural hearing loss associated with electrocardiograph anomalies. In this case study we report on a child affected with the Jervell and Lange‐Nielsen syndrome and suffering from congenital profound bilateral sensorineural hearing loss who had a cochlear implantation at age three. No surgical or cardiological complications were encountered and excellent post‐operative functional hearing and communicative outcomes were achieved. It is concluded that cochlear implantation could be safely performed in these patients, with the achievement of a very good outcome in functional hearing and communication.

Bilateral amplification for the elderly: are two aids better than one?

This paper reviews the advantages and disadvantages of bilateral amplification as opposed to unilateral hearing use for older persons with bilateral symmetric hearing loss. Binaural advantages, such as improved localization and speech recognition in noise, are presented as they pertain to the older population. In addition, contraindications, such as binaural interference, increased costs, cosmetic concerns, decreased manipulation skills, and additional hearing aid management issues, are discussed, A. case study is provided in which unilateral hearing aid fitting was more beneficial to a patient than two hearing aids. It is concluded that bilateral amplification should be attempted for all elderly patients with symmetric hearing loss, unless a contraindication is suspected.

Bilateral sensorineural hearing loss complicating basal skull fracture

Bilateral sensorineural hearing loss complicating basal skull fracture

Choanal stenosis, hypothelia, deafness, recurrent dacryocystitis, neck fistulas, short stature, and microcephaly: report of a case.

We report on a 11-year-old girl with bilateral choanal stenosis, hypothelia, hearing loss, recurrent dacryocystitis, neck fistulas, short stature, and microcephaly. Only three individuals with choanal atresia from a consanguineous family have been reported. One of the patients also had hypoplastic nipples, hypotonia, and delay in speech development. Similar clinical features were seen in two children reported by Greenberg [1987: Am J Med Genet 28:931-934] and Wilson et al. [1998: Am J Med Genet 75:220-222]. They were prenatally exposed to methimazole because of maternal Graves disease. Neck fistulas and microcephaly noted in our patient were not previously reported as features of the syndrome or in the patients prenataly exposed to methimazole. Our patient and those reported by Qazi et al. [1982: Am J Med Genet 13:413-416] most probably have a rare syndrome characterized by this distinctive combination of symptoms. Prenatal exposure to methimazole can cause a phenocopy of the syndrome, which was probably the case in the patients reported by Greenberg and Wilson et al.