1/10. klippel-trenaunay-weber syndrome with labyrinthine bony overgrowth and mixed hearing loss, a case report. Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by a triad of (1) capillary malformations, (2) venous malformations, and (3) soft tissue or bony hypertrophy. There exists one report of a patient with KTS and an associated sensorineural hearing loss. We describe an adolescent girl with KTS and AV fistulas who was found to have a bony overgrowth extending from the cochlea into the middle ear cavity and an ipsilateral mixed hearing loss. In both of these patients, there were obvious KTS deformities involving the face. We suggest conducting hearing screening in patients with KTS and obvious head involvement. ( info) |
2/10. odontoma of the middle ear: case report with 25-year follow-up. We report the 25-year follow-up on the first reported case of odontoma in the middle ear. diagnosis of odontoma had been made on the basis of radiography films that showed a middle ear mass with multiple toothlike areas of radiopacity. No clinical intervention was recommended. At 25-year follow-up, audiometry showed progressive mixed hearing loss on the affected side and mild ipsilateral sensorineural hearing loss. Computed tomography better characterized the discrete mass, which was shaped similar to a dental crown. To avoid jeopardizing cochlear and facial nerve function, no surgical intervention was pursued, and we recommended use of a BiCROS (bilateral contralateral routing of signal) hearing aid. ( info) |
3/10. Traumatic prepontine tension pneumocephalus--case report. OBJECTIVE: A case of prepontine tension pneumocephalus after temporal bone fracture is presented. CASE REPORT: An 8-year-old girl suffered a head injury due to a fall off her bicycle. She lost her consciousness, and when she was admitted to the local hospital the Glasgow coma Score (GCS) was 8/15 (eye opening: 2; verbal answer: 2; motor response: 4) and there was bleeding from the right ear. The patient's condition deteriorated rapidly and she needed intubation and ventilation. CT of the brain revealed large amount of air in the prepontine region, displacing the brainstem posteriorly. Patient was kept ventilated, meanwhile cerebrospinal fluid (CSF), as otorrhea appeared on the right side. CT was repeated 36 hours later, showing significantly less air in the prepontine area. The patient was weaned off the respirator, extubated and the level of consciousness improved. Later the patient developed meningitis, which was treated by systemic antibiotics with lumbar CSF drainage applied for five days. A high resolution CT scan of the petrous bone revealed a fracture crossing the middle part of the pyramid. Patient showed a full recovery except a right-sided mixed hearing loss. CONCLUSION: Rapid neurological deterioration following head injury can be a consequence of tension pneumocephalus. Prepontine pneumocephalus can be caused by minor fracture of petrous bone. High resolution CT is necessary to visualize minor fracture of the petrous bone. Conservative treatment may be satisfactory to treat tension hydrocephalus. ( info) |
4/10. Management of hemorrhagic high jugular bulb with adhesive otitis media in an only hearing ear: transcatheter endovascular embolization using detachable coils. A 51-year-old woman had a hemorrhagic high jugular bulb protruding into the posterosuperior part of the tympanic cavity in her left ear. This (only hearing) ear had severe adhesive otitis media resulting in repetitive hemorrhage from the jugular bulb. Therefore, treatment to stop the bleeding was required. Since invasive middle ear surgery in an only hearing ear would involve a high risk of hearing loss and massive bleeding, transcatheter endovascular embolization using detachable coils was selected for this special case; it safely blocked the blood flow and preserved the patient's hearing level. This is the first such report in the otosurgical field, showing that transcatheter endovascular embolization using detachable coils was quite effective in a difficult case of a hemorrhagic high jugular bulb with severe adhesive otitis media in an only hearing ear. ( info) |
5/10. CT findings of head and neck anomalies in lacrimo-auriculo-dento-digital (LADD) syndrome. We present computed tomography (CT) findings of a boy with lacrimo-auriculo-dento-digital (LADD) syndrome. Bilateral lacrimal glands were not detected and CT imaging of this abnormality in LADD syndrome has not been reported before. Despite suggestions from previous reports of multiple temporal bone abnormalities resulting in hearing losses in these patients, only minor temporal bone abnormalities were seen in our deaf-mute patient. Dysplasia of salivary glands was noted. CT findings of head and neck abnormalities in LADD syndrome are discussed. ( info) |
6/10. Sacculo-collic pathway dysfunction accompanying auditory neuropathy. CONCLUSIONS: In a patient with bilateral auditory neuropathy (AN), the vestibular-evoked myogenic potential (VEMP) was probably absent because of a neuropathy involving the inferior vestibular nerve and/or its end organ, the saccule. Our result can therefore be interpreted as a concomitant unilateral sacculo-collic neuropathy. We suggest the use of more precise terms to characterize AN patients with involvement of different parts of the inner ear and its innervations. We encourage detailed vestibular assessment in patients with AN in order to assess the co-existence of any symptomatic or asymptomatic vestibular disorder. Information such as that provided in this report will be valuable for clinicians caring for this group of patients. OBJECTIVE: AN is a disorder characterized by the absence or severe impairment of auditory brainstem responses in the presence of normal cochlear outer hair cell function as revealed by otoacoustic emissions (OAEs) and/or electrocochleography (ECoG). A variety of processes and etiologies are thought to be involved in its pathophysiology. In most literature reports the auditory profile of patients with AN is discussed. However, the extent of vestibular involvement, especially that involving the saccule, is not known. We performed vestibular tests to assess the status of the saccule in a patient with AN. MATERIAL AND methods: One patient with AN was studied. The patient was a right-handed 21-year-old female with chief complaints of hearing loss and speech perception difficulty. RESULTS: The auditory test results were consistent with the diagnosis of AN, i.e. absent auditory brainstem responses, moderate hearing loss, an inappropriately profound speech discrimination score and the presence of OAEs and measurable cochlear microphonics on ECoG. On neurological examination, gait and balance tests were normal. ice-water caloric testing induced a sensation of dizziness in both ears. Short tone-burst VEMPs showed no response on left-ear stimulation and a biphasic response with normal latency and amplitude on right-ear stimulation. ( info) |
7/10. Primary tuberculous petrositis. Tuberculous osteomyelitis of the temporal bone is a rare and dangerous entity that should be included in the differential diagnosis of infectious processes of the base of the skull. We present the case of an 11-year-old child who presented with diplopia, ear discharge and hearing loss. The radiological and histopathological findings revealed tuberculous otitis with osteomyelitis and an abscess in the petrous apex. The child responded to anti-tuberculous chemotherapy. The diagnosis and management of tuberculous osteomyelitis are discussed and a brief review of the literature is presented. ( info) |
8/10. Vertically oriented internal auditory canal in an 8-year-old with hearing loss. To report a unique orientation of the internal auditory canal and possible association with congenital hearing loss. Retrospective chart review of an 8-year-old Hispanic male with a mixed hearing loss. Uniquely abnormal orientation of the internal auditory canal in a patient with apparently normal cochleovestibular structures and a mixed hearing loss. A vertically oriented IAC is a rarely described anatomical anomaly of the temporal bone that may have associations with congenital hearing loss. ( info) |
9/10. A new syndrome of microtia with mixed type hearing loss, renal agenesis, and multiple skeletal anomalies. We report on a 17-year-old man who presented with unreported combination of right sided microtia and preauricular skin tag with conductive hearing loss, unilateral renal agenesis, partial syndactyly of forth and fifth metacarpals, multiple tarsal coalitions, absent toe, and hypoplastic tibia and fibula. Radiological and clinical findings did not match with the previously described syndromes with the type of anomalies seen in the case. We propose that this represents a new syndrome. ( info) |
| 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. ( info) |