Case report: Meniere's disease and otosclerosis-Different outcomes of the same disease?

The etiologies of Meniere's disease and otosclerosis are largely unknown. An association between these two diseases has been proposed on both a clinical and histopathologic basis but the causal relationship is controversial. In this paper we report two families in which both otosclerosis and Meniere's disease are inherited as independent phenotypes suggesting that the two diseases represent different outcomes of the same mutation. Thus the Meniere's disease occasionally seen in otosclerotic patients might not be caused by otosclerosis, but rather by a molecular defect leading to endolymphatic hydrops and/or clinical otosclerosis.     

Is microvascular compression of the vestibulocochlear nerve a cause of unilateral hearing loss?

OBJECTIVES: We sought to confirm earlier findings in the literature that microvascular compression of the vestibulocochlear nerve might cause unilateral sensorineural hearing loss. We measured the length and width of the internal auditory canal (IAC) to investigate a possible association between a narrow porus, the presence of an anterior inferior cerebellar artery (AICA) loop, and the development of a microvascular compression syndrome. METHODS: We performed a prospective blinded analysis of 167 magnetic resonance imaging scans of the cerebellopontine angle. The presence of an AICA loop was scored. We analyzed these 167 patients for unilateral sensorineural hearing loss, which was defined as an interaural difference of 20 dB at 1 frequency or 10 dB at 2 or more frequencies. Furthermore, the width and length of the IAC on magnetic resonance imaging were measured. RESULTS: An AICA loop was identified in 94% of the 167 patients. There were 196 type I loops, 106 type II loops, and 14 type III loops. Sixty-six patients had unexplained unilateral hearing loss. There was no association between type II and III vascular loops, the width of the IAC, and unilateral hearing loss (p > .05). CONCLUSIONS: In this study we found no association between the depth of extension of the AICA loop into the IAC and the presence of unilateral hearing loss.     

What is the effect of dizziness on the quality of life for patients with Meniere's disease?

OBJECTIVE: To evaluate the impact of dizziness on quality of life (QOL) in Meniere's disease (MD) patients. METHODS OF STUDY: Fifty definite MD patients were submitted to the Dizziness Handicap Inventory (DHI), in and out of crisis. The DHI scores were correlated to age, sex, race, disease duration, unilateral/bilateral labyrinth involvement, duration/frequency of vertigo attacks, vestibular and auditory function, aural fullness, tinnitus, imbalance and functional disabilities. Statistical analysis was performed by ANOVA. RESULTS: During crisis, all DHI aspects scores were statistically higher (p < 0.001) than out of crisis. Out of crisis, a statistically significant correlation was found between physical, functional and total scores and bilateral involvement. CONCLUSIONS: QOL impairment is worse during a Meniere's crisis. Out of crisis, QOL impairment is greater in patients with bilateral involvement.     

Rosai–Dorfman disease in the submandibular salivary glands of a pediatric patient

Neck mass is a common presentation in the pediatric patient. A case is discussed of a boy complaining of submandibular salivary gland enlargement, eventually leading to compressive dysphagia. Excisional biopsy yielded diagnosis of sinus histiocystosis with massive lymphadenopathy (Rosai Dorfman disease). Clinical findings, histopathology, and management of this rare disease are reviewed.     

Basaloid squamous carcinoma of the nasal cavity — a case report

Basaloid squamous carcinoma is a rare variant of squamous carcinoma and is known for its aggressive behavior. Its presentation in the nasal cavity is uncommon . A case of this tumor arising from the nostril and showing good response to concomitant radiotherapy and cisplatinum is presented.     

Streptomycin perfusion of the labyrinth in the treatment of Menière's disease: a modified technique

The ideal surgical procedure for Menière's disease would combine the high rate of vertigo control and the good hearing preservation of vestibular nerve section with the low morbidity of labyrinthectomy. Shea's technique of streptomycin perfusion of the labyrinth has been modified by making an additional opening into the posterior semicircular canal in an effort to limit the amount of streptomycin going into the cochlea. Seventeen patients with definite Menière's with poor hearing have had this procedure. Vertigo was controlled in 94% and the hearing preserved in 55%. Vestibular rehabilitation was not a problem. It is speculated that hearing preservation would be better if the procedure were not restricted to those with poor hearing. This method of destruction of the vestibular system carries the possibility of hearing preservation and maintains the possibility of cochlear implantation should this ever be required.     

Varied manifestations of Rosai-Dorfman disease and its management — a case report and review of literature

Rosai Dorfman disease or sinus histiocytosis is a rare, idiopathic, benign and self-limiting histiocytic proliferative disorder, usually seen in younger patients. It most commonly involves the cervical lymph nodes, with a predominant infiltration of sinusoidal histiocytes and classically presents with massive cervical lymhadenopathy. Extranodal disease occurs in about 43% of cases and produces different signs and symptoms depending upon its location. We report a case of this disorder in 22-year-old male with initial isolated involvement of bilateral nasal cavity and paranasal sinuses with subsequent involvement of cervical lymph nodes and skin. The clinical presentation, histologic characterstics, radiographic findings and treatment of the disease are discussed.     

Quality of life after gamma knife radiosurgery treatment in patients with a vestibular schwannoma: the patient’s perspective

This study evaluates the impact of gamma knife radiosurgery (GKRS) on the quality of life (QOL) of patients with a sporadic vestibular schwannoma (VS). This study pertains to 108 VS patients who had GKRS in the years 2003 through 2007. Two different QOL questionnaires were used: medical outcome study short form 36 (SF36) and Glasgow benefit inventory (GBI). Radiosurgery was performed using a Leksell 4C gamma knife. The results of the QOL questionnaires in relation to prospectively and retrospectively gathered data of the VS patients treated by GKRS. Eventually, 97 patients could be included in the study. Their mean tumor size was 17 mm (range 6–39 mm); the mean maximum dose on the tumor was 19.9 Gy (range 16–25.5 Gy) and the mean marginal dose on the tumor was 11.1 (range 9.3–12.5 Gy). SF36 scores showed results comparable to those for a normal Dutch population. GBI showed a marginal decline in QOL. No correlation was found between QOL and gender, age, tumor size, or radiation dose. Increased audiovestibular symptoms after GKRS were correlated with a decreased GBI score, and decreased symptoms were correlated with a higher QOL post-GKRS. In this study shows that GKRS for VS has little impact on the general QOL of the VS patient. However, there is a wide range in individual QOL results. Individual QOL was influenced by the audiovestibular symptoms. No predictive patient, tumor, or treatment factors for QOL outcome after GKRS could be determined. Comparison with microsurgery is difficult because of intra group variability.     

Functional outcome of treatment of Ménière's disease with the Meniett pressure generator

Conclusion. Use of the Meniett pressure generator in patients with active Ménière's disease (MD) did not affect labyrinthine function. Objective. To investigate whether application of the Meniett pressure generator in patients with active MD significantly affects their labyrinthine function. Material and methods. We measured hearing and labyrinth sensitivity in patients with established MD according to American Academy of Otolaryngology—Head and Neck Surgery (AAO-HNS) criteria at five different time periods: at baseline; before ventilation tube placement; before Meniett treatment; immediately after Meniett treatment; and 1 month after cessation of Meniett treatment. Results. A total of 32 patients met the inclusion criteria and were included in the study. They were graded as having definite MD (AAO-HNS stages 2 and 3). No statistically significant changes in labyrinthine function were found during Meniett treatment.     

Functional outcome of treatment of Ménière's disease with the Meniett pressure generator

CONCLUSION: Use of the Meniett pressure generator in patients with active Ménière's disease (MD) did not affect labyrinthine function. OBJECTIVE: To investigate whether application of the Meniett pressure generator in patients with active MD significantly affects their labyrinthine function. MATERIAL AND METHODS: We measured hearing and labyrinth sensitivity in patients with established MD according to American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) criteria at five different time periods: at baseline; before ventilation tube placement; before Meniett treatment; immediately after Meniett treatment; and 1 month after cessation of Meniett treatment. RESULTS: A total of 32 patients met the inclusion criteria and were included in the study. They were graded as having definite MD (AAO-HNS stages 2 and 3). No statistically significant changes in labyrinthine function were found during Meniett treatment.     

Leiomyoma of the external auditory canal—a case report and brief review of the literature

Leiomyoma usually originates from the uterus and alimentary tract, but rarely from the soft tissue of the head and neck. It is extremely rare that leiomyoma appears in the external auditory canal. A 47-year-old male with left external auditory canal leiomyoma presented with conductive hearing impairment and post-auricular swelling. Tumor excision was successful via a retroauricular approach. The pathological examination demonstrated a vascular leiomyoma without malignant change. To date, including our case, only three cases of external auditory canal leiomyoma have been reported. The tumor occurs mainly in adult males aged around 50 and should be included in the differential diagnoses for external auditory canal tumor. The proper treatment of choice is complete excision. In case of an external auditory canal tumor, even if it is believed to be benign before surgery, precise pathologic examination is required to exclude the possibility of malignancy.