Reports of patients with concurrent middle and inner ear anomalies are rare. These patients present a surgical challenge for cochlear implantation. The surgical risk must be weighed against the predicted benefit of the patient\'s hearing outcome and subsequent development of speech and language as well as their quality of life.
Thirteen-year-old boy presented to the Otology clinic for auditory rehabilitation options. He has mild developmental delay, is non-verbal and communicates via American Sign Language. He was born with bilateral aural atresia and never wore amplification. On exam he has grade 1 microtia and complete ear canal atresia bilaterally. His behavioural hearing test shows profound sensorineural hearing loss of both ears. The computed tomography scan shows bilateral underdeveloped and completely opacified mastoid and middle ear, complete bony atresia of the ear canals, and an under-partitioned cochlea with poorly defined modiolus, among other abnormalities. The patient and his family were counselled on the available options as well as the need for any further studies.
Counselling of patient and family.
While there have been reports in the literature of performing cochlear implantations in patients with a concurrent atresia and cochlear dysplasia, these were patients whose degree of inner ear anomalies was relatively minor and their prognosis of a good audiological outcome was favourable. The presented case is that of a patient for whom the surgical approach to the cochlea alone would be difficult. More importantly, his quality of life would not significantly improve in light of the predicted limited hearing and language development outcomes, given the severity of his inner ear abnormalities, limited communication abilities, prolonged period of deafness and developmental delays.

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OBJECTIVE: To present the first reported case of intraneural direct cochlear nerve stimulation in a human being.
METHODS: This is a case report.
RESULTS: A 23-year-old patient with bilateral progressive hearing loss associated with bilateral complete semicircular canal aplasia and ossified cochleas underwent cochlear implantation. During surgery, a patent cochlear lumen could not be found, and the array was positioned in the internal auditory canal adjacent to the cochlear nerve. Against our expectations, an assiduous rehabilitation and frequent fitting adjustments have led to a word recognition score, in open set speech with lip reading, of 18/25 and acceptable frequency discrimination.
CONCLUSIONS: We are aware that this was an anomalous use of the cochlear implant, and it is not our aim to suggest a new indication for cochlear array positioning. However, this case shows that auditory perception, to some degree, can be obtained with intraneural direct cochlear nerve stimulation.

BACKGROUND: Lyme disease is the most common vector-borne disease in the United States and is caused by infection with the spirochete Borrelia burgdorferi. In children, neuroborreliosis usually presents as peripheral facial nerve palsy and lymphocytic meningitis and only rarely is associated with cranial polyneuritis.
METHODS: We present a 15-year-old with tinnitus, hearing loss, and facial nerve palsy in the setting of acute, severe right arm pain and a several week history of malaise and headache. Lumbar puncture was notable for lymphocytic pleocytosis. Serologic testing demonstrated positive Lyme antibody and a positive immunoglobulin M Western blot. Immunofluorescent assay of cerebrospinal fluid was also positive for anti-Lyme immunoglobulin M. Audiologic testing revealed mixed, right-sided hearing loss. Neuroimaging demonstrated cranial polyneuritis and right-sided cochlear inflammation. The patient was treated with parenteral ceftriaxone with resolution of his symptoms at close follow-up.
CONCLUSIONS: Neuroborreliosis with radiculopathy, lymphocytic meningitis, and cranial polyneuritis is a rare presentation of pediatric Lyme disease. Additionally, cochlear inflammation along with cranial nerve VIII inflammation may contribute to hearing loss in patients with neuroborreliosis.

OBJECTIVE: To study the steps involved in definitive evaluation and successful management of patients with congenital perilymph fistula presenting with recurrent meningitis.
METHODS: Case series with chart review.
METHODS: Tertiary care center.
METHODS: The case records of 11 patients (12 ears) treated for congenital perilymph fistula presenting with recurrent meningitis were reviewed to ascertain their clinical, radiological, and intraoperative features and outcome following surgery.
RESULTS: Most patients presented after at least 3 episodes of meningitis (range, 2-10 episodes). Ipsilateral hearing loss was present in 9 of 12 ears, with normal hearing in 3 patients. High-resolution computed tomography and/or magnetic resonance imaging scanning of the temporal bone contributed to the diagnosis in 75% of cases but was normal in 3 cases (25%). Oval window and round window defects were the most common (66.7% and 63.6%, respectively). Four ears (33.3%) had more than 1 defect. The unusual presentations included 2 patients who presented in adulthood, a patient with a defect in the medial wall of the attic, and 3 patients with normal radiological findings. Follow-up ranged from 1 to 11 years (median, 2 years). There were 2 failures following simple fistula closure with cessation of symptoms after vestibular obliteration. No patient was readmitted with recurrent meningitis after definitive surgery.
CONCLUSIONS: Up to 25% of patients with recurrent meningitis secondary to congenital perilymph fistula may have normal audiological and radiological assessment necessitating exploratory tympanotomy. Vestibular obliteration, rather than simple fistula closure, prevents recurrence.

BACKGROUND: Temporal bone fracture is a common complication of high-energy cranial trauma. Labyrinth involvement is rare, but there is a risk of perilymphatic rupture that is often underestimated on initial clinical examination due to the predominance of neurological and/or somatic symptoms.
METHODS: A patient presented with overlooked perilymphatic fistula, decompensated by hyperpressure effort due to poorly adapted management.
CONCLUSIONS: Following a review of the literature on post-traumatic pneumolabyrinth, overall management (from diagnosis to treatment, via prevention advice) was analyzed. A constructive critique of the patient\'s treatment was thus made.
CONCLUSIONS: We argue for a systematic management protocol in cranial trauma with temporal bone fracture, comprising ENT examination, millimetric-scale cross-sectional imaging of the fracture site, and standardized counseling to prevent late complications.

Multiple semicircular canal dehiscences are clinical entities characterised by vestibular and cochlear symptoms induced by enhanced sensitivity of labyrinthine receptors due to a multiple bone defect of the otic capsule. The case is presented of a 38-year-old male with bilateral posterior semicircular canal dehiscence associated with unilateral (right) superior semicircular canal dehiscence. The man suffered from vestibular (recurrent Tullio Phenomenon or sound-induced vertigo) and cochlear symptoms (persistent aural fullness associated with mixed hearing loss and disabling tinnitus).

A 38-year-old male presented with a meningioma within the internal auditory canal (IAC) manifesting as rapidly progressive hearing loss over a period of one month. He had a 2-year history of tinnitus. Magnetic resonance imaging revealed a 10-mm intracanalicular tumor, which was surgically resected by a retrosigmoid lateral suboccipital approach. The histological findings showed meningothelial meningioma. The patient had no facial palsy after surgery, but his cochlear function did not recover. Common symptoms of IAC meningiomas are tinnitus and hearing loss, but rapidly progressive hearing loss is very rare. IAC meningioma is rare but should be taken into consideration as a cause of rapidly progressive hearing loss.

OBJECTIVE: To investigate the hypothesis of cochlear and retrocochlear damage in scrub typhus, using evoked response audiometry.
METHODS: Prospective, randomised, case-control study.
METHODS: The study included 25 patients with scrub typhus and 25 controls with other febrile illnesses not known to cause hearing loss. Controls were age- and sex-matched. All subjects underwent pure tone audiometry and evoked response audiometry before commencing treatment.
RESULTS: Six patients presented with hearing loss, although a total of 23 patients had evidence of symmetrical high frequency loss on pure tone audiometry. Evoked response audiometry found significant prolongation of absolute latencies of wave I, III, V, and wave I-III interpeak latency. Two cases with normal hearing had increased interpeak latencies. These findings constitute level 3b evidence.
CONCLUSIONS: Findings were suggestive of retrocochlear pathology in two cases with normal hearing. In other patients, high frequency hearing loss may have led to altered evoked response results. Although scrub typhus appears to cause middle ear cochlear and retrocochlear damage, the presence of such damage could not be fully confirmed by evoked response audiometry.

We report here on a case of congenital cholesteatoma that extended into the internal auditory meatus and cochlea. A 17-year-old boy underwent surgery for a very large cholesteatoma, which was discovered behind an intact tympanic membrane. Pure tone audiometry revealed an unresponsive ear. High resolution temporal bone computed tomography showed perilabyrinthine extension with its absence in the vestibular area, and destruction of the bony cochlea at the basal turn, the tegmen and the posterior cranial fossa. Magnetic resonance imaging revealed no intracranial extension. Surgical exploration of the middle ear and the mastoid cavity revealed that both the vestibule and the basal turn of the cochlea were filled with a noninfected cholesteatoma. The cholesteatoma extended into the internal auditory meatus through translabyrinthine destruction; it extended into the basal turn of the cochlea through the infralabyrinthine route. The bony segment of the facial nerve canal demonstrated near total dehiscence. The cholesteatoma was removed by the transotic approach. Congenital cholesteatoma is characterized by no specific history. Therefore, early detection of this malady can be challenging, but it is important to prevent such complications as were observed in this reported case.