Aneurysmal bone cyst of the temporal bone associated with reversible hemifacial paralysis

Aneurysmal bone cyst (ABC) is an uncommon lesion of the temporal bone (TB), with only 20 cases reported. Facial paralysis is a rare complication (2 cases); however, no cases have been reported with preoperative reversal of paralysis. We report a 60-year-old man with a history of remote head trauma, who presented with serious otitis media and right hemifacial paralysis, which resolved with nonsurgical therapeutic measures. Magnetic resonance imaging and computed tomography showed a destructive and expansile lesion of the TB. The lesion was surgically removed, and ABC was diagnosed histologically. The patient had an uneventful recovery and demonstrated no recurrence at 1 year of follow-up. This report presents an unusual presentation of ABC in the TB, with a review of the clinical, radiological, pathological, and therapeutic features of this entity.     

Auditory and facial nerve dysfunction in patients with hemifacial microsomia

BACKGROUND: Hemifacial microsomia (HFM) is a common craniofacial disorder characterized by a wide spectrum of anomalies, including conductive hearing loss due to external and middle ear deformities. However, the prevalence of sensorineural hearing loss (SNHL) as well as facial nerve dysfunction is underappreciated. OBJECTIVE: To determine the frequency of auditory and facial nerve dysfunction and its relationship to more severe forms of bilateral HFM. DESIGN: Retrospective medical record review to characterize the clinical severity of HFM and the prevalence and nature of the associated auditory and facial nerve dysfunction. SETTING: Center for Craniofacial Anomalies at the University of California, San Francisco, Medical Center. PATIENTS: Ninety-nine pediatric patients with HFM evaluated at the University of California, San Francisco, Medical Center. MAIN OUTCOME MEASURES: The prevalence of SNHL and facial nerve dysfunction in this patient population and any associations between these 2 characteristics. RESULTS: Hearing loss was present in 74 (75%) of 99 patients, with a conductive component in 73 patients. Sensorineural hearing loss was present in 11 patients ( 11%), with mixed hearing loss in most patients. Fourteen patients required rehabilitation with auditory amplification. Nearly a quarter of the patients (22 [22%] of 99) had facial nerve dysfunction, but only 1 patient had facial palsy on the same side as the SNHL. There was a statistically significant association between having auricular abnormalities and conductive hearing loss or SNHL (P = .30 and .80, respectively). However, there was no statistically significant association between bilateral HFM and the occurrence of either SNHL or facial paralysis, nor was there an association between auditory and facial nerve dysfunction. CONCLUSIONS: Sensorineural hearing loss and facial nerve dysfunction are common in HFM. These findings have important implications in the treatment of patients with HFM.     

Autosomal dominant sensorineural hearing loss. Pedigrees, audiologic findings, and temporal bone findings in two kindreds

We report the clinical and otopathologic findings in three persons from two kindreds affected with adult-onset autosomal dominant progressive sensorineural hearing loss. The primary pathologic change is a deposit of acid polymucosaccharide ground substance in the cribrose areas; in the spiral ligament, limbus, and spinal lamina of the cochlea; and in the stroma of the maculae and cristae. These deposits obstruct the channels that accommodate the dendritic nerve fibers to the auditory and vestibular sense organs. The end result is strangulation and degeneration of dendrites followed by retrograde neuronal degeneration in association with varying degrees of atrophic change in the sense organs.     

Audiometric abnormalities in hemifacial spasm

One-hundred forty-three patients with hemifacial spasm (HFS) as a primary disorder were evaluated pre- and postoperatively by pure-tone and speech audiometry, determination of thresholds for the acoustic middle ear reflex, and recording of auditory brainstem responses. We found an unusual 'notch' in the pure-tone audiograms of 16 patients (11%) and a low-frequency up-sloping pure-tone threshold in another 17 patients (12%). Because such abnormalities occur rarely, it may be assumed that they are related to these patients' primary disorder. All of the patients underwent microvascular decompression to relieve their spasms, and it was confirmed during surgery that in all patients the seventh cranial nerve was compressed at its root entry zone by vascular structures. It is therefore assumed that the abnormality of pure-tone threshold function noted in 23% of the patients with HFS was caused by compression of the auditory nerve by the same vessel as that which compressed the facial nerve. Since the changes in the pure-tone threshold occurred in the mid- and low-frequency range, it is assumed that low-frequency fibers constitute the outer part of the auditory nerve at its entrance into the human brainstem.     

Histopathologic study of temporal bone and eustachian tube in oculoauriculovertebral spectrum

Bilateral temporal bone specimens from a 21-month-old girl and a left temporal bone-eustachian tube (ET) specimen from a full-term female newborn, both with oculoauriculovertebral spectrum, were studied histopathologically. The external and middle ears demonstrated severe anomalies, similar to those of previous reports describing the histopathologic findings of this syndrome. In addition, despite having a normal auricle, the 21-month-old child had bilateral hypoplastic cochleas as seen in Mondini dysplasia. The newborn had several anomalies of the ET, including a widely opened cartilaginous portion of the ET lumen and absence of the lateral lamina of the ET cartilage. We discuss the implications of the observed anomalies with regard to developmental and clinical issues.     

Major and minor temporal bone abnormalities in children with and without congenital sensorineural hearing loss

OBJECTIVE: To determine the extent of correlation between sensorineural hearing loss (SNHL) and abnormal temporal bone anatomy in children. DESIGN: Axial and coronal high-resolution computed tomographic scans of the temporal bones of 247 children (494 ears) aged 2 months to 15 years with and without SNHL were blindly reviewed. The presence or absence of mild or severe cochlear dysplasias, vestibular dysplasias, and an enlarged vestibular aqueduct (VA) were recorded. The width of the VA was measured. The height, width, and length of the internal auditory canal (IAC) were measured, and abnormalities were described as narrow, widened, or bulbous. Clinical information was then reviewed to determine the presence or absence of a congenital syndrome and/or SNHL, and historical factors that might be responsible for SNHL. MAIN OUTCOME MEASURE: The relationship between radiographic findings and SNHL. RESULTS: One hundred thirteen patients (185 ears) had SNHL. Significant abnormal temporal bone anatomy in children with vs without SNHL included major cochlear and vestibular dysplasias (17% vs 0%; P<.001), enlarged VA (>2 mm) (5% vs 0%; P<.001), and narrow IAC (< or =2 mm) (4% vs 1%; P=.03). The average IAC width (4.85 vs 5.02 mm), height (4.39 vs 4.62 mm), and length (11.22 vs 11.44 mm) were not statistically different between children with vs without SNHL. In children with vs without SNHL, neither a widened (0.5% vs 3.6%) nor a bulbous (9% vs 8%) IAC was seen more often in children with SNHL. In ears with SNHL, the presence of a congenital syndrome significantly increased the risk of cochlear and vestibular abnormalities of the temporal bone (45% vs 14%; P<.001), including IAC abnormalities (30% vs 2%;P<.001), which overall were more commonly seen in children with (20%) vs without (3%) a congenital syndrome regardless of the presence of SNHL. No children with an enlarged VA had a congenital syndrome. CONCLUSIONS: Well-established temporal bone abnormalities such as cochlear and vestibular abnormalities and a grossly enlarged vestibular aqueduct are significantly found in children with SNHL. A narrow IAC is found more often in children with vs without SNHL. No significant correlation is found between SNHL and radiographic findings of a widened or bulbous IAC. In children with a congenital syndrome, more IAC abnormalities were seen, regardless of the presence of SNHL. In children with SNHL, the presence of a congenital syndrome increases the likelihood of a cochlear or vestibular abnormality.     

Use of reconstructed,non-orthogonal plane,high-resolution computed tomography of the temporal bone in the planning of temporal bone surgery

OBJECTIVES: To demonstrate the utility of high-resolution computed tomography (HRCT) reconstructed in non-orthogonal planes in the planning of temporal bone surgery. STUDY DESIGN: Qualitative and quantitative comparison of in vivo anatomic measurements between orthogonal and non-orthogonal plane reformatted HRCT of the temporal bone. METHODS: HRCT data of 10 normal temporal bones were reconstructed two-dimensionally in two non-orthogonal planes. Parallel to the plane defined by the superior semicircular canal (defined as transverse) as well as perpendicular to the plane defined by the superior semicircular canal (defined as longitudinal). This was done using commercially available software. Sixteen surgically important relationships between neural, vascular and/or bony structures were measured and analyzed. RESULTS: Quantitatively, wide variations were obtained in the measurements obtained from images of both non-orthogonal planar orientations. These variations were not age or sex dependent. Qualitatively, the images obtained highlight the anatomy of the temporal bone and skull base as it will be encountered during otologic surgery, since the surgical visual axis is along the long axis of the temporal bone. CONCLUSIONS: The reformatted images in non-orthogonal planes were superior to the axial and coronal series because they condensed critical relationships into a single perspective which promoted an intuitive understanding of the surgical approach. These reconstructed images show wide differences in quantitative measurements between surgically relevant landmarks. The protocol can be easily implemented in the clinical setting and is a potentially valuable educational tool. We recommend that non-orthogonal reconstructed images be routinely included with orthogonal temporal bone HRCT scans.     

The role of audiologic evaluation in progressive audiologic tinnitus management

Progressive Audiologic Tinnitus Management (PATM) is based on the premise that tinnitus is managed most efficiently using a hierarchy of clinical services that address different levels of need. PATM includes five levels of management: (a) triage; (b) audiologic evaluation; (c) group education; (d) tinnitus evaluation; and (e) individualized management. This article provides an overview of PATM and focuses on the procedures that make up the Level 2 Audiologic Evaluation. The evaluation is conducted to assess the potential need for medical, audiologic (hearing loss, tinnitus, hyperacusis), and/or mental health services. The Tinnitus Handicap Inventory, Hearing Handicap Inventory, and Tinnitus and Hearing Survey are used to differentiate effects of tinnitus and hearing loss. If indicated, patients are interviewed with the Tinnitus-Impact Screening Interview. Patients requiring amplification receive hearing aids. Often, management of hearing loss at Level 2 addresses any problems that were attributed to the tinnitus, which obviates further tinnitus-specific intervention.     

Acromegaly and the temporal bone

Acromegaly is a chronic disease of middle life resulting from excessive secretion of growth hormones by the acidophil cells of the anterior pituitary. The typical clinical features include enlargement of the skull, thorax, hands and feet. Recently, three patients with acromegaly have been operated upon for active otologic disease. In spite of massive mastoid cortex bone, the structures of the otic capsule were found to be of normal dimensions and in the usual relationships.     

乙状窦源性耳鸣颞骨气化程度的CT研究

目的 探讨颞骨气化程度及含气状态在乙状窦源性耳鸣发生中的作用.方法 分析手术证实的29例乙状窦源性耳鸣和116例正常人颞骨CT图像.结果 正常组男性右侧颞骨气化Ⅱ度3例,Ⅲ度19例,Ⅳ度36例;左侧气化Ⅱ度5例,Ⅲ度12例,Ⅳ度41例.女性右侧颞骨气化Ⅱ度5例,Ⅲ度10例,Ⅳ度43例;左侧气化Ⅱ度6例,Ⅲ度9例,Ⅳ度4...     

Usher's syndrome, temporal bone pathology

The histological findings in the right temporal bone of a 65-year-old deaf and blind man are presented. The subject suffered from the autosomal recessive Usher's syndrome, as did 2 of his 5 siblings. They are the offspring of a consanguineous marriage. This man died from an intra-abdominal hemorrhage. Within 3 h after death the temporal bones were donated for study and were processed for histopathological examination.     

Histiocytosis of the temporal bone

Histiocytosis is a rare disease of unknown aetiology which commonly affects the head and neck region. In the ear it can closely mimic acute and chronic ear disease leading to a confusion in diagnosis. It can be easily misdiagnosed unless a high index of clinical suspicion is maintained. We present a case of Letterer Siwe disease of the temporal bone which presented with bilateral mastoid abscesses.