Cholesteatoma: recognition and management

A cholesteatoma is an abnormal collection of viable and desquamated squamous epithelium in the middle ear or mastoid air spaces. Early recognition and treatment are important. Unlike chronic middle ear effusion or recurrent otitis media, a cholesteatoma inexorably destroys the middle and inner ear. Delayed diagnosis increases the chance of otologic complications, such as permanent hearing loss, permanent balance dysfunction, infection and facial paralysis. Initial management includes careful cleansing of the ear and topical and oral antibiotics. Definitive treatment requires surgical excision.     

砧骨搭桥与钛质人工听骨植入在鼓室成形术中的疗效观察

目的:探讨鼓室成形术中自体砧骨搭桥与钛质人工听骨植入的临床疗效。方法:回顾性分析运用自体砧骨搭桥行鼓室成形术的66例非中耳胆脂瘤患者,以及运用钛质人工听骨植入行鼓室成形术的30例中耳胆脂瘤患者的临床资料。术后气-骨导差(ABG)≤20 dB HL视为手术成功标准。随访6~34个月,平均21个月,观察术后并发症,并行听力学疗效分析。结果:96例患者术后6~8周均获干耳,未见外耳道狭窄、肉芽生长、胆脂瘤复发等不良事件;所有病例鼓膜修补膜生长良好,未见再次穿孔和听骨赝复物移位、脱出。自体砧骨组病例手术成功率为77.2%(51/66),3例术后听力改善不明显,1例继发分泌性中耳炎,行鼓膜切开置管后痊愈。钛质人工听骨组病例手术成功率为76.6%(23/30),1例术后听力改善不明显。结论:对于锤骨及镫骨完整、活动度良好,砧骨有不同程度破坏或中断的非中耳胆脂瘤患者,在鼓室成形术中利用自体砧骨搭桥行听骨链重建可获得较好的听力改善;对于锤骨及镫骨底板完整、活动度良好,砧骨、镫骨板上结构有不同程度破坏或中断的中耳胆脂瘤患者,在鼓室成形术中选用钛质人工听骨置植入行听骨链重建可获得较好的听力改善。     

Unusual presentation of glomus tympanicum tumour: New bone formation in the middle ear

The objective of this study is to increase awareness of the rare presentation, diagnostic difficulties and management of glomus tympanicum of the middle ear. A 49 years old male, with a background of hypertension and epilepsy, presented with a two month history of left sided conductive hearing loss, pulsatile tinnitus and headache. Clinically and radiologically a diagnosis of glomus tympanicum was made. Intraoperatively, extensive osteogenesis of the middle ear resulting in ossicular fixation and erosion was found. This patient required a two stage operation for full clearance of disease. A stapedectomy drill was used to drill off the bony overgrowth surrounding the ossicles resulting in improved hearing thresholds and full clearance of the disease at two year follow up. Glomus tympanicum can result in new bone formation in the middle ear with resultant ossicular fixation and conductive hearing loss. This can be effectively treated surgically with restoration of hearing.     

Unilateral sudden sensorineural hearing loss with vertigo as a first symptom of bilateral internal auditory canal metastases: a case report

There have been relatively few reports of bilateral internal auditory canal metastases of asymptomatic primary pulmonary adenocarcinoma presenting as unilateral sudden sensorineural hearing loss and vertigo. We report a case of a 60-year-old male patient who complained of sudden hearing loss in the right ear and vertigo. Upon a physical examination, no definite neurological signs or nystagmus were observed. Pure-tone audiometry showed deafness in the right ear at all frequencies and high-frequency sensorineural hearing loss in the left ear. The video head impulse test suggested bilateral vestibulopathy. Magnetic resonance imaging of the brain (with gadolinium contrast) revealed bilateral internal auditory canal enhancement and a variable-sized nodular and peripheral-enhancing lesion in the cerebrum and the right cerebellum. A computed tomographic and bronchoscopic biopsy identified asymptomatic primary pulmonary adenocarcinoma in the left upper lobe of the lungs. This is a rare report of bilateral internal auditory canal metastases in an asymptomatic patient with primary pulmonary adenocarcinoma who initially presented with symptoms of unilateral sudden sensorineural hearing loss with vertigo.     

Tubotympanic catarrh in childhood. Diagnosis and therapy

Tubotympanic catarrh (otitis media with effusion) is a long-known disease. Today it has become a "fashionable disease", but is extremely important as the most frequent cause of hearing loss in children. The key to the disease is functional disability of the auditory tube. Therapeutic pitfalls and a significant lack of compliance on the part of the patient have definitely led to an increase in otitis media with effusion. We are sure that better diagnostic facilities for the detection of hearing loss in children has made the "silent syndrome" more prevalent. Therapy must aim to cure tubal dysfunction as soon as possible to enable clearance of the middle ear secretion and ventilation of the tympanon, in order to avoid the feared complications of adhesive otitis and atypical cholesteatoma. Prophylaxis is better than cure.     

Mouse model of enlarged vestibular aqueducts defines temporal requirement of Slc26a4 expression for hearing acquisition

Mutations in human SLC26A4 are a common cause of hearing loss associated with enlarged vestibular aqueducts (EVA). SLC26A4 encodes pendrin, an anion-base exchanger expressed in inner ear epithelial cells that secretes HCO3- into endolymph. Studies of Slc26a4-null mice indicate that pendrin is essential for inner ear development, but have not revealed whether pendrin is specifically necessary for homeostasis. Slc26a4-null mice are profoundly deaf, with severe inner ear malformations and degenerative changes that do not model the less severe human phenotype. Here, we describe studies in which we generated a binary transgenic mouse line in which Slc26a4 expression could be induced with doxycycline. The transgenes were crossed onto the Slc26a4-null background so that all functional pendrin was derived from the transgenes. Varying the temporal expression of Slc26a4 revealed that E16.5 to P2 was the critical interval in which pendrin was required for acquisition of normal hearing. Lack of pendrin during this period led to endolymphatic acidification, loss of the endocochlear potential, and failure to acquire normal hearing. Doxycycline initiation at E18.5 or discontinuation at E17.5 resulted in partial hearing loss approximating the human EVA auditory phenotype. These data collectively provide mechanistic insight into hearing loss caused by SLC26A4 mutations and establish a model for further studies of EVA-associated hearing loss.     

开放式乳突根治术联合乳突腔填充治疗胆脂瘤中耳炎的临床可行性

目的分析开放式乳突根治术联合乳突腔填充治疗胆脂瘤中耳炎的临床可行性。方法将本院收治的100例胆脂瘤中耳炎患者随机分为观察组和对照组,各50例。两组均接受开放式乳突根治术治疗,观察组在此基础上联合乳突腔填充治疗。比较两组的治疗效果。结果观察组的术后干耳时间、术腔上皮化时间、切口愈合时间、住院时间均短于对照组(P<0.05)。观察组的外耳道形态正常率及鼓膜愈合率均高于对照组(P<0.05)。观察组治疗后的气导听力、骨导听力、气骨导差及CSOM-QOL各项评分均优于对照组(P<0.05)。结论开放式乳突根治术联合乳突腔填充治疗胆脂瘤中耳炎的效果显著,可改善患者听力水平及外耳道形态,提高鼓膜愈合率及生活质量,值得临床应用。     

Hörscreening an Kindergärten: Ergebnisse einer Evaluierungsstudie

OBJECTIVE: This study aimed to evaluate the hearing screening of pre-school children at nursery schools in Tyrol, Austria. METHODS AND SAMPLE: 47 nursery schools with a total of 2199 enrolled children participated in the study. At the screening, the children were presented a series of tones at frequencies 0.5 kHz (25dB), 1 kHz, 2 kHz, 3 kHz, and 4 kHz (20 dB each) from portable audiometers. The tones were presented over headphones for each ear separately and at irregular intervals. Failure to respond to any of the frequencies was considered failure of the screening. Parents were then advised in written form to have the child examined by an ENT-specialist. RESULTS: 1832 individuals were screened (coverage: 83% of nursery school children; corresponding to at least 63% of all Tyrolean children aged 3 to 5 years). Of these, 390 failed the test (referral rate: 21% of all screened). Examination through an ENT-specialist occurred with 217 children, and this confirmed the positive test in 139 children (hit rate: 64%). In most cases, a temporary conductive hearing loss due to external or middle ear problems (glue ear, tube dysfunction, cerumen, otitis media) was diagnosed. A sensorineural hearing loss was found in 4 children (in 3 of them bilateral). The need for therapy was recognized in 81 children (4% of all screened). CONCLUSION: Pre-school hearing screening identifies children with ear and hearing problems that need therapeutical intervention. Although the hearing problems are mostly of a temporary nature, some may require monitoring over some period. Also some children with permanent sensorineural hearing loss may be detected through this measure. Hearing screening is an efficient means of assessing ear and hearing problems in pre-school children. However, the follow-up rate needs to be improved for optimizing the efficacy.     

Restoring hearing symmetry with two cochlear implants or one cochlear implant and a contralateral hearing aid

With today's technology and the demonstrated success of cochlear implantation, along with expanded candidacy criteria, the opportunity to provide optimal hearing to both ears for individuals with severe-to-profound hearing loss is greater than ever. This article reviews the advantages of binaural hearing and the disadvantages of hearing with only one ear or hearing with two ears with significantly different sound thresholds. A case study is presented that demonstrates the benefit of bimodal hearing (i.e., a cochlear implant [CI] in one ear and a contralateral hearing aid [HA]) in a nontraditional CI candidate with asymmetrical hearing thresholds. Then, selected studies in adult recipients who use a CI and contralateral HA or who use two CIs are summarized. The data overall demonstrate that bilateral CI recipients, traditional bimodal recipients, and nontraditional bimodal recipients experience substantial binaural hearing advantages, including improved speech recognition in noise, localization, and functional everyday communication. These results indicate that bilateral stimulation of the auditory system through a CI and contralateral HA or two CIs is beneficial and should become standard clinical practice.     

Topical ocular drug delivery to inner ear disease and sinusitis

Oral steroids are the main therapy for sensorineural deafness. We present the rare case of a patient whose hearing loss associated with inflammation of the inner ear and chronic sinusitis were improved with topical steroid therapy. A 68-year-old male presented with scleritis in the left eye, inflammation of the inner ear and chronic sinusitis. He received oral prednisolone 10 mg/d. However, the oral prednisolone was discontinued due to severe side effects. Topical administration of 0.1% betamethasone sodium phosphate improved the scleritis and incidentally also relieved his symptoms of recurrent otitis and sinusitis after several days. Audiometry revealed recovered acoustic sensation in the right ear, from 50 dB to 20 dB, and in the left ear from 70 to 35 dB with 1,000 Hz. Topical ocular drug delivery of steroids may be effective for inner ear disease and sinusitis in patients with systemic side effects to oral steroids.     

IL-1β inhibition in autoimmune inner ear disease: can you hear me now?

Clinical vignette: A 51-year-old man with right-sided sudden hearing loss presents to the otology clinic. He has a 4-year history of episodic vertigo of several hours’ duration and fluctuating, progressive sensorineural hearing loss in his left ear. The vertigo attacks have not occurred for the last 18 months, and the left ear hearing is consistently poor. The patient’s right ear hearing has dropped in the last 36 hours. MRI imaging of brain and temporal bone are normal. A 2-week “burst and taper” of oral prednisone is administered with no effect. Over the next 3 months, serial audiograms show rapidly progressive loss of threshold and word recognition scores on the right side. A trial of high-dose prednisone (60 mg/d for 30 days) results in full recovery of the right ear hearing and substantial improvement in the left ear. As the prednisone dose is slowly tapered over several months, the hearing drops again.The patient above is likely suffering from autoimmune inner ear disease (AIED), a rare form of sensorineural hearing loss (SNHL). In 1979, McCabe provided the first clinical evidence that SNHL could have an autoimmune component (1), supporting the hypothesis put forth by Lehnhardt (2). Despite multiple confirmatory reports that have refined the clinical characterization of AIED, the pathogenesis of this very uncommon condition remains vague and controversial, in large part because there are no universally accepted diagnostic criteria or tests. A loose consensus has gradually arisen that AIED presents as an idiopathic, rapidly progressive, bilateral SNHL. AIED patients exhibit a loss of hearing that occurs over a period of weeks to months, too slow to be considered “sudden” SNHL and too fast to be classified as age-related or other genetically determined degenerative SNHL. Serial audiometry demonstrates progression, which is usually monotonic but may fluctuate. Typically the ears progress asynchronously, often years apart. In many cases, deafness in the first ear may be congenital or the result of some identifiable cause prior to rapid hearing loss in the second ear, raising the possibility of AIED. This rapidly progressive asynchronous hearing loss alone is considered sufficient by some to make an AIED diagnosis, whereas others require evidence of steroid responsiveness.     

Sound preconditioning therapy inhibits ototoxic hearing loss in mice

Therapeutic drugs with ototoxic side effects cause significant hearing loss for thousands of patients annually. Two major classes of ototoxic drugs are cisplatin and the aminoglycoside antibiotics, both of which are toxic to mechanosensory hair cells, the receptor cells of the inner ear. A critical need exists for therapies that protect the inner ear without inhibiting the therapeutic efficacy of these drugs. The induction of heat shock proteins (HSPs) inhibits both aminoglycoside- and cisplatin-induced hair cell death and hearing loss. We hypothesized that exposure to sound that is titrated to stress the inner ear without causing permanent damage would induce HSPs in the cochlea and inhibit ototoxic drug–induced hearing loss. We developed a sound exposure protocol that induces HSPs without causing permanent hearing loss. We used this protocol in conjunction with a newly developed mouse model of cisplatin ototoxicity and found that preconditioning mouse inner ears with sound has a robust protective effect against cisplatin-induced hearing loss and hair cell death. Sound therapy also provided protection against aminoglycoside-induced hearing loss. These data indicate that sound preconditioning protects against both classes of ototoxic drugs, and they suggest that sound therapy holds promise for preventing hearing loss in patients receiving these drugs.     

The perforated tympanic membrane.

Tympanic membrane perforations typically result from trauma or acute otitis media. Most perforations do not cause more than a mild conductive hearing loss, aural fullness and mild tinnitus. Blood, purulent secretions and other debris should be carefully suctioned out of the canal and the perforation size and location described. Irrigation and pneumatic otoscopy should be avoided. A history of vertigo, nausea and vomiting and an audiogram showing a conductive hearing loss of more than 30 dB suggest disruption of the ossicular chain. Profound sensorineural loss may signify inner ear nerve damage. Mastoid radiographs and computed tomographic scans may be useful in cases of significant trauma and infection. Most small perforations resolve spontaneously. The affected ear should be kept dry. Oral and topical antibiotics may be prescribed for perforations related to acute otitis media. Otolaryngologic referral may be necessary to evaluate traumatic perforations associated with vertigo or significant hearing loss, perforations from chronic otitis media or perforations from acute otitis media that do not heal within one month.     

儿童感音神经性耳聋中先天性内耳畸形的临床特征

目的：分析在儿童感音神经性耳聋（sensorineural hearing loss,SNHL）中先天性内耳畸形患儿的影像学及临床特征。方法：回顾分析860例SNHL患儿中因听力障碍（109例）、外耳畸形（3例）、面部异常（13例）来就诊的125例（225耳）患儿的临床资料。结果：109例（87.2%）患儿因听力障碍就诊,母亲妊娠早期有感冒史52例（41.6%）。166耳（73.8%）极重度耳聋,累及双侧100例（80%）。颞骨高分辨率CT或磁共振成像（MRI）显示其中同时合并外耳及中耳畸形3耳,合并中耳畸形13耳。Michel畸形2例（4耳）,耳蜗不发育1例（1耳）,共同腔畸形10例（14耳）,不完全分隔-I型2例（2耳）,耳蜗前庭发育不良2例（2耳）,不完全分隔-II型（Mondini畸形）19例（29耳）。累及前庭导水管扩大91例（170耳）（75.6%）,半规管畸形30例（46耳）（20.6%）,内听道畸形35例（49耳）（21.7%）。结论：前庭导水管扩大是儿童SNHL中最常见的内耳影像学异常。患儿多为双侧重度听力损失或全聋。对于先天性内耳畸形的患儿行高分辨率CT检查非常重要。     

Bone anchored hearing aids (B.A.H.A.)

Semi-implantable bone conduction hearing aids of the type Bone Anchored Hearing Aids (B.A.H.A.) consist of an external vibrator hearing aid that is attached to a titanium screw implanted in the mastoideal bone behind the ear by means of a transcutaneous connector. In this way sound waves are transmitted directly to the bones of the skull, from where they are conducted to the cochlea. The primary indication for a semi-implantable bone conduction hearing aid is hearing loss due to conduction failure that is not surgically correctable. This may be caused, e.g. by ear canal atresia and other malformations of the external auditory meatus and of the middle ear, by chronic ear infections or by persistent high grade conduction hearing impairment after radical mastoidectomy. Today B.A.H.A.'s largely replace the less convenient conventional bone conducting hearing aids as hearing glasses or hearing bands, in which a vibrator is pressed against the skull. In conduction hearing impairment on both ears B.A.H.A.'s can be implanted bilaterally and thus binaural hearing with sound source localisation are made possible. A novel application is in cases of unilateral deafness, where the device is implanted on the side of the deaf ear and the amplified sounds are transmitted through the skull to the side of the hearing ear. Here it serves as a substitute for a CROS hearing aid. In adults the implantation is performed under local anesthesia as an out-patient procedure. Implantation may be undertaken under general anesthesia on children after two years of age. In Berne, B.A.H.A. bone conduction hearing aids have been implanted in 112 patients (ages 2-82 years) in the past 13 years. The operations have proved simple and without intra-operative complications. The long-term post-operative incidence of complications was low. The transcutaneous screw caused only isolated cases of transient skin reactions or local infections. In 7% of cases, rejection of the implant or a traumatic loosening of the screw required a reimplantation procedure. Over 90% of patients reported themselves satisfied with their partially-implantable B.A.H.A. hearing aids.     

Biological therapy for the inner ear

Biological therapy for the inner ear has the potential to revolutionise the treatment of sensorineural hearing loss, the most common form of deafness. Progress in the molecular understanding of hearing and hearing loss, combined with advances in the fields of both gene and cellular therapy for the inner ear, is providing a robust foundation from which clinical translation is plausible. Potential areas of interest in gene therapy and its preclinical application to deafness are reviewed, and experimental progress that has occurred in cellular therapy for the inner ear is examined.     

Biological therapy for the inner ear

Biological therapy for the inner ear has the potential to revolutionise the treatment of sensorineural hearing loss, the most common form of deafness. Progress in the molecular understanding of hearing and hearing loss, combined with advances in the fields of both gene and cellular therapy for the inner ear, is providing a robust foundation from which clinical translation is plausible. Potential areas of interest in gene therapy and its preclinical application to deafness are reviewed, and experimental progress that has occurred in cellular therapy for the inner ear is examined.     

The unilateral stapes gusher

A stapes gusher is an alarming situation occurring during stapes surgery for mixed hearing loss. It is characterized by the sudden profuse flow of cerebrospinal fluid in the middle ear and external ear canal after opening the vestibule of the inner ear. An adult patient with unilateral stapes gusher is described; he was operated on both ears with a good hearing result on both sides. The stapes on the side with the cerebrospinal fluid leak was malformed, especially in the posterior crus--a finding which may suggest the possibility of a stapes gusher. The small-hole stapedotomy is a safe technique when dealing with a profuse cerebrospinal fluid leak, and it enables the closure of the leak with additional tamponade of the oval niche and the achievement of a good hearing result.     

Current status and prospects of gene therapy for the inner ear

Inner ear diseases are common and often result in hearing disability. Sensorineural hearing loss is the main cause of hearing disability. So far, no effective treatment is available although some patients may benefit from a hearing aid equipped with a hearing amplifier or from cochlear implantation. Inner ear gene therapy has become an emerging field of study for the treatment of hearing disability. Numerous new discoveries and tremendous advances have been made in inner ear gene therapy including gene vectors, routes of administration, and therapeutic genes and targets. Gene therapy may become a treatment option for inner ear diseases in the near future. In this review, we summarize the current state of inner ear gene therapy including gene vectors, delivery routes, and therapeutic genes and targets by examining and analyzing publications on inner ear gene therapy from the literature and patent documents, and identify promising patents, novel techniques, and vital research projects. We also discuss the progress and prospects of inner ear gene therapy, the advances and shortcomings, with possible solutions in this field of research.     

Middle ear problems in Aboriginal school children cause developmental and educational concerns

An epidemiological study was carried out in the year 2000 and sought to measure the occurrences of middle ear disease and hearing loss within school aged (4 years to 12 years) Aboriginal children. A number of the local schools and preschools in Coraki and Lismore with a high percentage of Aboriginal students were selected in an effort to identify service gaps regarding essential hearing screenings and assessments. A total of 185 (370 ears) Aboriginal children aged 4 years to 12 years were examined from four schools and three preschools. This examination included otoscopy, tympanometry and audiometry. Data were collected as each child was tested and this was then entered into a computer database on returning to the work place. Results indicated that 61.08% of these children had middle ear problems of some type. Unilateral hearing loss of 30 dB or greater was found in 10.80% of children, bilateral hearing loss of 30 dB and greater was found in 22.16%, and perforation of tympanic membranes in 3.24%. Suggestions are made in relation to the need for ongoing training of Aboriginal Community Audiometrists to provide community, school and preschool screening programs together with health related promotional activities to minimise the occurrences of ear infections.