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At least 217 records · Page 12Linked to original sources

Usher's syndrome. Ophthalmic and neuro-otologic findings suggesting genetic heterogeneity.

The conditions of 70 patients with Usher's syndrome were studied by ophthalmic and neuro-otologic examinations. Two distinct clinical and presumed genetic types were discernible on the basis of differences in hearing impairment, vestibular sensitivity, and, to a lesser extent, deterioration in retinal photoreceptor function. Distinguishing these two types has relevance for both diagnosis and genetic counseling of patients with Usher's syndrome.

Adolescent↗

Otologic manifestations of Wolf-Hirschhorn syndrome.

OBJECTIVE: To determine if haploinsufficiency for chromosome 4p16.3 in Wolf-Hirschhorn syndrome (WHS) is associated with cochlear hearing loss. DESIGN: Case series. SETTING: Tertiary care center. PATIENTS: Six patients with WHS were identified through a database and charts were retrospectively reviewed. MAIN OUTCOME MEASURES: Presence of sensorineural hearing loss as assessed by brainstem auditory evoked response. RESULTS: One of the 6 patients had sensorineural hearing loss. Three of the 6 patients had chronic otitis media with effusion and underwent bilateral tympanostomy tube placement; 2 of these 3 had cleft lip and palate, and 1 had a bifid uvula. One of the 6 patients had spontaneous nystagmus. Five of the 6 patients had preauricular and/or auricular abnormalities. CONCLUSIONS: More than 25 genes for nonsyndromic hereditary hearing impairment have been mapped. One of these genes, DFNA6, was identified through linkage analysis of a family with dominant, progressive, low-frequency sensorineural hearing loss. DFNA6 maps to chromosome 4p16.3, a region that is partially deleted in patients with WHS. In our series, we identified the second patient with WHS in the literature with bilateral sensorineural hearing loss. The incidence and type of otologic findings are consistent with those reported in the literature. Analysis of patients with chromosomal rearrangements represents one strategy toward identifying candidate genes for genetic hearing impairment.

Audiometry, Evoked Response↗

Acoustic reflex test in neuro-otologic diagnosis. A review of 24 cases of acoustic tumors.

Acoustic and other tumors of the cerebellopontine angle are not uncommon, and they usually have otologic symptoms or findings. Early diagnosis depends on a high index of suspicion and a thorough evaluation of patients with suspected tumors. This is a report of 24 cases selected from a group of 184 surgically confirmed tumors. Had the cases been evaluated only on the basis of pure tone or speech audiometry or on the basis of plain roentgenograms, the diagnosis might not have been made. Audiological examination should include the acoustic reflex test. The results of this test indicated a retrocochlear problem (absence or decay of the reflex) in 19 of 24 cases (80%). The test was positive in 11 of 12 patients with normal or near normal hearing and in seven of eight patients with normal x-ray films.

Adolescent↗

The epidermis of the drumhead in some otologic conditions.

In a study of the behavior of the keratin layer of the drumhead epidermis in some otologic conditions, a marker dye was applied to the tympanic membrane, and migration and/or desquamation of the dotted spots observed. Decelerated migration with desquamation in situ was observed during the quiescent phase of recurrent otitis externa, with or without myringitis. The same findings were observed in most cases of chronic secretory otitis media and its sequelas (retraction pockets, atrophic membranes, and scarring). The remnants of drumhead with keratotic and hyperplastic changes in the middle ear cleared themselves of the dye very slowly.

Adolescent↗

Cerebral cortical veins in otologic surgery.

Proximity of the cerebral cortical veins to the overlying dura predisposes them to surgical injury. Labbé's vein, draining a major portion of the posterior temporal and inferior parietal lobes, occurs on the left in 77% and on the right in 66% of patients. Interruption of this vein during otologic surgery may produce devastating neurologic consequences. In this regard, the roentgenographic anatomy of cerebral cortical venous drainage is reviewed. Technical treatment of the temporal dura should include those techniques familiar to the neurosurgeon that ensure the pretection of the temporal lobe and its superficial venous drainage.

Adult↗

Intravenous histamine in otologic practice. Side effects in 2,347 administrations.

Intravenous (IV) histamine has been used extensively at the Otologic Medical Group, Inc, Los Angeles, for 30 years in the treatment of various conditions in which vasospasm was thought to play a part in the cause. We report the findings on 2,347 IV histamine administrations to 622 patients during a 33-month period. Dizziness was the indication for treatment in half of the patients. Sensorineural hearing impairments was the diagnosis in an additional 44%, and the majority of these had had a sudden impairment. Side effects were uncommon, never serious, and bore no relationship to the results of the histamine skin test or the patient's general medical problem. The IV histamine treatment resulted in improvement in symptoms in 36%, and almost half of those improvements were immediate.

Adult↗

Otologic effects of interferon beta serine in experimental rhinovirus colds.

To evaluate the efficacy and otologic effects of recombinant interferon beta serine for experimental rhinovirus colds, 38 healthy adults received nasal drops of recombinant interferon beta serine, 12 x 10(6) U, or placebo three times daily for 4.3 days beginning 36 hours after infection. Illness rates and severity did not differ between the groups, but the frequency of virus shedding was reduced on the fourth (37% vs 74%) and sixth (11% vs 42%) postchallenge days in recipients of recombinant interferon beta serine. Abnormal eustachian tube function in at least one ear was identified by the inflation-deflation test during 44% of observations in 13 infected recipients of recombinant interferon beta serine compared with 62% of observations in five placebo recipients. Tympanometry revealed middle-ear pressure abnormalities (less than -50 or greater than 20 mm H2O) in at least one ear during 18% of observations in recipients of recombinant interferon beta serine compared with 38% of observations in placebo recipients. These results suggest that antiviral therapy may alter the course of middle-ear dysfunction associated with experimental colds.

Administration, Intranasal↗

Otologic complaints in temporomandibular joint syndrome.

Complaints of diminished hearing or reduced speech discrimination frequently accompany temporomandibular dysfunction. There is no consensus as to the mechanism of their occurrence or the alteration of these symptoms with the treatment. We studied 12 subjects with internal derangement of the temporomandibular joint (treated surgically) and nine subjects with myofascial pain disorder (treated medically), and we found no difference between the groups in pretreatment audiometric findings or in their degree of otologic symptoms. Similarly, there were no differences in posttreatment audiometric measures and there were no significant treatment effects. Furthermore, there was no correlation between subjects' complaints of reduced hearing sensitivity or discrimination and audiometric results. The apparently significant reduction in symptoms experienced by some subjects in the absence of audiometric change suggests the operation of unmeasured factors in their response to treatment.

Adolescent↗

Otologic manifestations in congenital velopharyngeal insufficiency.

A study of ear disease in children with congenital velopharyngeal insufficiency (VPI) was undertaken. A retrospective chart analysis of 80 cases of VPI formed the basis of this review. Thirty-seven percent of the children had evidence of middle ear disease, with middle ear effusion and recurrent otitis media being the most frequent findings. Thirty-one percent had abnormal audiograms, with 20% having a conductive or mixed hearing loss. Overall, 46% of the children had otologic manifestations, indicating the need for the pediatrician and the otolaryngologist together to be actively involved in the evaluation and treatment of this group of children, starting in early infancy.

Adenoidectomy↗

Otological complications of herpes zoster.

Otological complications of varicella-zoster virus (Ramsay Hunt syndrome) include facial paralysis, tinnitus, hearing loss, hyperacusis (dysacousis), vertigo, dysgeusia, and decreased tearing. Cranial nerves V, IX, and X are often affected. Gadolinium-enhanced magnetic resonance imaging demonstrates enhancement of the geniculate ganglion and facial nerve. These manifestations are identical to Bell's palsy but are more severe and carry a graver prognosis. Eight percent of Bell's palsy patients eventually are diagnosed as "zoster sine herpete." A new case of Ramsay Hunt syndrome will occur every 52 minutes, compared to every 10 minutes for a new case of Bell's palsy.

Ear Diseases↗

Recent advances in neuro-otology.

Four advances in the field of neuro-otology are described: (1) auditory brain-stem response audiometry, (2) electroneuronography, (3) the cochlear implant, and (4) the infratemporal fossa approach to the skull base. Auditory brain-stem response audiometry is an accurate method for the early detection of hearing impairment and early diagnosis of acoustic neurinomas. Electroneuronography accurately assesses the degree of injury to the facial nerve, thereby improving management of facial nerve lesions. The cochlear implant restores the sensation of sound to totally deaf patients. The infratemporal fossa approach is used to treat extensive lesions of the skull base.

Audiometry↗

Unusual otologic manifestation of chronic lymphocytic leukemia.

The first reported case of a patient with chronic lymphocytic leukemia, who 14 years after diagnosis developed bilateral symmetrical ear lobe swelling secondary to lymphocytic infiltrates and its management, is presented. The various otologic and specific skin manifestations of leukemia are discussed and the literature reviewed. The temporal bone findings take the form of leukemic infiltrations, inflammation, and hemorrhage. These may present as a red or thickened tympanic membrane, hemotympanum, exudates in the middle ear, acute otitis media, mastoiditis, conductive or sensori-neural hearing loss, dizziness, vertigo or facial paralysis. The specific skin manifestations take the form of nodules, papules, infiltrations, plaques, ulcerations and exfoliative erythroderma. These may present as skin lesions most commonly in the head and neck area, rarely presenting in the auricle and ear canal.

Aged↗

Neuro-otologic manifestations of sarcoidosis.

Sarcoidosis is an idiopathic disease which presents in anatomic areas of concern to otorhinolaryngologists. It can cause dysfunction of both auditory and vestibular systems. In patients known previously to have sarcoidosis, this disease should be seriously considered. In patients presenting with otologic disorders and associated facial nerve paralysis or other neuropathies, uveitis, granulomatous meningitis or diabetes insipidus, sarcoidosis should be suspected. An examination of the eyes as well as a chest X-ray is imperative. Sudden and fluctuating neurosensory hearing loss has been reported. There is a great need for the study of temporal bones from sarcoidosis patients.

Adult↗

Microscopic characteristics of round window problems in otology.

Round window membrane ruptures and perilymph leakage can present the complication or concomitant of sudden deafness and barotrauma. Pathological involvement of the round window membrane can occur in many otological diseases such as otosclerosis, otitis media, tumors, etc. Microscopic manipulations might result in an inadvertent round window membrane lesion and cochlear deafness. In recent years purposeful surgical lesions, including procedures for vertigo and cochlear implant surgery, are being made in the round window membrane. Examples of pathology involving the round window in human temporal bones are described. To understand better the clinical features of early round window rupture in humans,, controlled punctuate lesions were made in 36 chinchilla ears and studied sequentially. Round window membrane healing occurred as early as 3 days post-lesion, and was complete in all ears but 1 of the combined 7, 9 and 11-day group. Healing occurred primarily as a result of middle ear epithelial ingrowth and mesothelial cell reaction in the adjacent perilymphatic space of the scala tympani. These cellular events are described and discussed from an histological and clinical point of view.

Adult↗

Microsurgery in otology and laryngology: a review of developments during the past thirty years.

The operating microscope was originally introduced to provide better visualization and illumination for otologic surgery. The successful use of this instrument in the fenestration operation for otosclerosis rapidly led to its application to reconstructive procedures in chronic suppurative otitis media. Later, it became evident that the operating microscope also provided advantages in the treatment of inner ear disorders and in the removal of acoustic Schwannomas; a considerable reduction in morbidity and an improved prognosis have resulted from application of microsurgery to these conditions. In addition, new photographic and television equipment, used in combination with microsurgery, has greatly improved the standards of teaching and the recording of pathologic information.

Humans↗

Ionomer-based bone substitute in otologic surgery.

During post-set hardening the self-curing bone substitute Ionocem develops a solid bond with the adjacent bony tissue, leaving no empty spaces. The fully matured material can be fixed to bone with freshly mixed cement or it may be used as a blank, e.g. an ossicular implant (Ionos ossicle). After insertion of 945 alloplastic middle ear prostheses over a period of 4.5 years, the take-rate was 94%. In some patients revision surgery became necessary, in 50% of cases because of prosthesis dislocation. A granular version of the cement (Ionogran) was implanted in 46 ears for obliteration of mastoid cavities and showed complete mucosal overgrowth within a maximal period of 3 months. Posterior canal wall reconstruction with the self-curing bone substitute was done in 74 patients, with revisions required in 12 cases because of persistent epithelial deficits in the external ear canal or epitympanic retraction. Overall results showed that the ionomer-based cement was a useful substitute for bone in reconstructive otologic surgery.

Animals↗

Pure-tone hearing thresholds in otologically healthy 5-year-old children in Finland.

We measured the pure-tone air and bone conduction hearing of 359 randomly selected otologically normal urban preschool children in Finland at the average age of 5.2 years. Children with otoscopically verified middle ear pathology or abnormal impedance audiometry were not included in this sample. The mean air conduction thresholds varied from 16.6 dB at 0.125 kHz to 6.6 dB at 2 kHz, and the mean bone conduction thresholds from 6.0 dB at 0.25 kHz to 0.7 dB at 4 kHz. The pure-tone average (of air conduction thresholds at 0.5, 1 and 2 kHz) of all the ears was 7.6 dB. The distribution of single air conduction hearing thresholds at the frequencies from 0.25 kHz to 4 kHz showed that 66%-75% were at the 5-10 dB level.

Auditory Threshold↗

Magnetic resonance imaging based classification of anatomic relationship between the cochleovestibular nerve and anterior inferior cerebellar artery in patients with non-specific neuro-otologic symptoms.

In this study, we aimed to assess anatomical relationship between the anterior inferior cerebellar artery (AICA) and cochleovestibular nerve (CNV) in patients with non-specific cochleovestibular symptoms using magnetic resonance imaging (MRI). One-hundred and forty patients with non-specific neuro-otologic symptoms were assessed using cranial and temporal MRI. Classification was performed according to four different types of anatomical relationship observed between the AICA and CVN. In type 1 (point compression), the AICA compresses only a limited portion of the CVN. In type 2 (longitudinal compression), the AICA approaches the CVN as both traverse parallel to each other. In type 3 (loop compression), the vascular loop of the AICA encircles the CVN. In type 4 (indentation), the AICA compresses the CVN so as to make an indentation in the nerve. The anatomical relationship between the CVN and AICA was encountered in 19 out of 140 (13.6%) patients (20 ears). The VCC was unilateral in 18 patients (94.7%) and bilateral in one patient (5.3%). There was no other vascular structure causing VCC to the CVN except for vertebral artery that was seen in 2 out of 140 patients (1.4%). These were unilateral cases. There were tinnitus, vertigo or dizziness, hearing loss, and both hearing loss and vertigo in 5 (25%), 13 (65%), 1 (5%) and 1 (5%) ears of 20 patients, respectively. There was no relationship between the cochleovestibular symptoms and type of compression (p>0.05). Neurovascular relationship between the CVN and AICA can be imaged properly using MR and MR based classification may help reporting this relationship in a standard way. Although, MR images can show the anatomical relationship accurately, diagnosis of vascular conflict should not be based on imaging findings alone.

Adolescent↗