1/323. The leaking labyrinthine lesion resulting from direct force through the auditory canal: report of five cases. The leaking labyrinthine lesion is treated by conservative methods or surgical procedures. With respect to the stapes, the surgical treatment is controversial. Five cases of middle ear injuries accompanying oval window rupture are reported herein. In each case, direct force through the auditory canal damaged not only the ossicular chain but also the oval window. Initial symptoms were sudden hearing loss with significant conductive disturbance and various degrees of unsteadiness. Spontaneous horizontal nystagmus directed toward the uninvolved ear was observed in each case. Tympanic cavities were promptly explored under general anesthesia and oval window injuries were confirmed. In each case, the damaged stapes was temporally removed from the oval window. Perilymphatic leakage was recognized in each case. Two patients had subluxation of the stapes with a paucity leakage. Three had complete luxation of the stapes with a relatively huge oval window fistula. Disrupted oval windows were repaired with temporalis muscle fascial grafts that were inserted under the middle ear mucosae surrounding the oval windows. The stapes were replaced in the repaired oval windows, and the ossicular chains were reconstructed without artificial grafts. Vestibular dysfunctions disappeared within 7 days, and satisfactory audiologic results were obtained in each case. ( info) |
2/323. Fibrous dysplasia of the temporal bone and maxillofacial region associated with cholesteatoma of the middle ear. Fibrous dysplasia of the temporal bone is a rare disease which may lead to progressive stenosis of the external auditory canal and the development of cholesteatoma. We present a case in which minimal symptoms were present despite a massive temporal bone fibrous dysplasia. cholesteatoma resulted most probably secondary to external auditory canal stenosis. Retroauricular fistula developed as a result of destructive effect of cholesteatoma, that influenced previous diagnosis and treatment of this clinically silent disease. ( info) |
3/323. Minimal, progressive, and fluctuating hearing losses in children. Characteristics, identification, and management. Referring to specific types of hearing loss as "minimal" or "mild" seems to imply that their effects are equally mild or negligible. A growing body of literature, however, supports the notion that such losses can have a significant impact on the communicative and educational development of young children. Although OME is considered a common childhood ailment, mounting evidence suggests that it is not always benign and may contribute to significant educational and communicative difficulties in some young children when accompanied by conductive hearing loss. Even very mild bilateral and unilateral SNHL seems to contribute to problems in the areas of social and emotional function, educational achievement, and communication in some children. Because these hearing losses are so mild, they may not be immediately recognized as the source of such difficulties. The purpose of this report is to heighten the general pediatrician's awareness of the significance of even very mild or minimal hearing losses in children. As the gatekeepers for children's health care, pediatricians are typically the primary recipients of parental expressions of concern and the initiators of evaluations or referrals to address such. ( info) |
4/323. External acoustic meatus cholesteatoma. cholesteatoma is a condition usually affecting the middle ear and mastoid, but in certain cases it can be found in the external ear canal. It usually affects elderly people and presents with chronic otorrhoea and dull otalgia. The disease can progress to extensive mastoid destruction. Small lesions can be treated with regular microscopic debridement in out-patients while larger lesions require surgery to eradicate the cholesteatoma sac. We present 2 cases of external ear cholesteatoma to add to the less than 50 cases reported to date. ( info) |
5/323. Proximal symphalangism and congenital conductive hearing loss: otologic aspects. OBJECTIVE: Results of stapedectomy are reported in a Belgian 26-year-old woman and two Dutch brothers having the proximal symphalangism syndrome (McKusick 18580). STUDY DESIGN: case reports are presented. A review of the results of ear surgery for congenital conductive hearing loss in this syndrome is given. SETTING: The Belgian patient was treated in a general hospital. The Dutch patients were treated in a university hospital, which was a tertiary referral center. patients: patients were referred to have an evaluation of their hearing impairment. INTERVENTION: Based on the syndromal diagnosis and based on routine audiometric tests, a congenital ossicular fixation was considered to be the cause of the hearing loss. By exploratory tympanotomies, this was confirmed. Reconstructive procedures including stapedotomy were performed. MAIN OUTCOME MEASURES/RESULTS: Long-term audiometric data are presented to evaluate the outcome of the surgical interventions. CONCLUSIONS: Congenital stapes ankylosis eventually combined with a congenital fixation of the short process of the incus in the fossa incudis, causing the congenital conductive hearing loss. Surgical intervention is very successful in most reported cases, but negative side effects are incidentally found as well. ( info) |
6/323. Jugular bulb dehiscence in achondroplasia. Jugular bulb dehiscence--complete absence of a roof over the jugular bulb--is a rare malformation, probably present in <<< 1% of the general pediatric population. Of 126 children with achondroplasia evaluated in the Midwest Regional Bone Dysplasia Clinic, four and probably five, were identified as having such dehiscence (at least 3.2% of the children assessed). Identifying this increased incidence in achondroplasia is of some clinical relevance, particularly including risk of difficult to control bleeding at myringotomy. It may also present as otherwise unexplained hearing loss, tinnitus and self audible bruits in these children. ( info) |
| beckwith-wiedemann syndrome is a rare genetic overgrowth syndrome presenting with organomegaly, abdominal wall defects, macroglossia, and postnatal hypoglycemia. Head and neck manifestations of this abnormality include flame nevus of the forehead and characteristic sulci of the ear lobe. We present a 7-year-old child with beckwith-wiedemann syndrome and a rare finding of conductive hearing loss on both sides due to congenital malleus and stapedial fixation. Small fenestra stapedotomy and mobilization of malleus fixation in the epitympanum improved the child's hearing. The bony fixation of the malleus and stapes is explained as atavism of the processus anterior mallei and peripheral lamina stapedialis in embryological development. ( info) |
8/323. A patient-benefit evaluation of unilateral congenital conductive hearing loss presenting in adulthood: should it be repaired? OBJECTIVE: To evaluate subjective and objective outcomes of surgical therapy in congenital conductive hearing loss (HL) presenting in adulthood. The impact of hearing restoration on quality of life (QOL) for these patients may differ from those with acquired HL. STUDY DESIGN: Retrospective review of five patients treated surgically. methods: Standard audiometry was completed. patients completed a subjective questionnaire as well as subjective (open-set) questions. A seven-point Likert scale was used for closed-set responses with 1 representing the worst possible outcome and 7 being best. RESULTS: The average four-frequency improvements in pure-tone average and air-bone gap were 18 dB (range, 7-29 dB) and 16 dB (range, 2-30 dB), respectively. An average score was obtained for each closed-set question. The highest-scoring items were for overall improvement of QOL (average score, 6), followed by perceived improvement in hearing (average score, 5.6). Other positive responses were improved ability to use the telephone (four of five patients) and improved localization of sound (four of five patients). Negative subjective responses included prolonged dysequilibrium in two of five, prolonged adjustment period in three, and dysgeusia in one patient. CONCLUSION: Important components of outcome are likely to escape our review if only objective measures are used to evaluate surgical outcomes. These results suggest that improved hearing in this population provides specific improvements in lifestyle and is perceived as a significant improvement in overall QOL. ( info) |
9/323. Greater superficial petrosal nerve neurinoma. Case report. The authors report a case of middle cranial fossa neurinoma arising from the left greater superficial petrosal nerve in a 21-year-old woman who presented with a left-sided otitis media that chronically recurred over a period of 5 years. On examination, the patient had a left-sided mild conductive hearing impairment and a slight disturbance in tear secretion on the left side, with sensory disturbance in the left palate. Three-dimensional computerized tomography scans clearly demonstrated the enlargement of the foramen lacerum and foramen ovale, and heavily T2-weighted magnetic resonance images revealed the tumor's location along with the course of the greater superficial petrosal nerve and its extension into the tympanic cavity. Following complete surgical excision of the tumor and tympanoplasty via a middle cranial fossa approach, the patient retained useful hearing without facial palsy. ( info) |
10/323. Traumatic ossicular chain discontinuity--report of two cases. In addition to hemotympanum and traumatic eardrum perforation, traumatic ossicular chain discontinuity should also be considered in the differential diagnosis of conductive hearing impairment resulting from head injury. The most common form of these ossicular chain lesions following head injury is incudostapedial joint (I-S joint) separation. We successfully managed two patients with I-S joint separation resulting from head injury through exploratory tympanotomy with ossiculoplasty within the recent 2 years. Both were young females who had sustained head injury resulting from traffic accident with the sequelae of persistent hearing impairment. They both gained significant hearing improvement postoperatively. ( info) |