Posterior petrous face meningiomas presenting with Ménière’s-like syndrome: a case series and review of the literature

OBJECTIVE Ménière’s disease is an inner ear disorder classically characterized by fluctuating hearing loss, tinnitus, and aural fullness accompanied by episodic vertigo. While the pathogenesis of Ménière’s remains under debate, histopathological analyses implicate endolymphatic sac dysfunction with inner ear fluid homeostatic dysregulation. Little is known about whether external impingement of the endolymphatic sac by tumors may present with Ménière’s-like symptoms. The authors present a case series of 7 patients with posterior fossa meningiomas that involved the endolymphatic sac and new onset of Ménière’s-like symptoms and review the literature on this rare clinical entity. METHODS A retrospective review of patients undergoing resection of a posterior petrous meningioma was performed at the authors’ institution. Inclusion criteria were age older than 18 years; patients presenting with Ménière’s-like symptoms, including episodic vertigo, aural fullness, tinnitus, and/or hearing loss; and tumor location overlying the endolymphatic sac. RESULTS There were 7 cases of posterior petrous face meningiomas involving the vestibular aperture presenting with Ménière’s-like symptoms. Imaging and intraoperative examination confirmed no cranial nerve VIII compression or labyrinthine artery involvement accounting for audiovestibular symptoms. Of the 7 patients in the series, 6 experienced significant improvement or resolution of their vertigo, and all 7 had improvement or resolution of their tinnitus after resection. Of the 5 patients who had preoperative hearing loss, 2 experienced improvement or resolution of their ipsilateral preoperative hearing deficit, whereas the other 3 had unchanged hearing loss compared to preoperative evaluation. CONCLUSIONS Petrous face meningiomas overlying the endolymphatic sac can present with a Ménière’s syndrome. Early recognition and microsurgical excision of these tumors is critical for resolution of most symptoms and stabilization of hearing loss.

Case Report: Ménière's Disease-Like Symptoms in 22q11.2 Deletion Syndrome

The 22q11.2 deletion syndrome (22q11.2DS), caused by a microdeletion on the long arm of chromosome 22, is characterized by congenital heart disease, hypoparathyroidism, immunodeficiency, developmental delay, and velopharyngeal insufficiency. Anatomic malformations of the middle and inner ears are frequently present, leading to high prevalence of hearing impairment. We present a first case of 22q11.2DS showing fluctuating hearing loss with recurrent vertigo attacks, resembling Ménière's disease. A 38-year-old male known to have 22q11.2DS developed recurrent vertigo, tinnitus, and fluctuating hearing loss in the left ear during a 10-year follow-up period. During vertigo attack, he had spontaneous left-beating nystagmus with downbeat components, but bithermal caloric and video head impulse tests showed normal vestibulo-ocular reflex functions. Sequential pure tone audiograms demonstrated fluctuating sensorineural hearing loss (SNHL) in both ears, which finally progressed to permanent hearing loss in the left ear. Computed tomography imaging of the temporal bone exhibited bilaterally malformed lateral semicircular canals, and delayed 3D-FLAIR sequences revealed cochlear endolymphatic hydrops with dilation of the scala media in the left ear. This case shows that acute vertigo with SNHL can be one of the audiovestibular presentations in 22q11.2DS caused by disturbance of endolymphatic flow.

Meniere Disease: A Case Report

Background: Meniere’s disease is a clinical disorder defined as the idiopathic syndrome of endolymphatic hydrops. Meniere’s disease has a prevalence of about 200 cases per 100,000 people in the United States. Prevalence increases linearly with increasing age, especially over 60 years. Men and women are estimated to be proportional in the number of cases. The etiology of Meniere’s disease is currently known to be caused by intrinsic factors and extrinsic factors. Symptoms of Meniere’s disease include recurrent spontaneous episodic vertigo, fluctuating hearing loss, ear fullness, and tinnitus. Treatment of Meniere’s disease can be divided into pharmacological therapy and non-pharmacological therapy. In some cases, complaints can be reduced to 80-90 percent. Case Report: A woman, aged 65 years, complained of constant ringing in her right ear, feeling full and accompanied by decreased hearing in the right ear, spinning dizziness that is not related to changing position, especially when she is tired, ringing in the ear, a feeling of fullness in the ear since 3 months ago. Head MRI results were normal. The patient was treated with HCT 1×50 mg, prednisone 1×80 mg for 7 days, KSR 3×1 tablet, betahistine 2×24 mg. Patients experience improvement in complaints even though the complaints do not disappear completely Conclusion: The diagnosis of Meniere’s disease is established by clinical symptoms, namely recurrent spontaneous episodic vertigo, fluctuating hearing loss, ear fullness, and tinnitus. Appropriate therapy in Meniere’s disease can reduce complaints and improve the patient’s quality of life.

Fluctuating Sensorineural Hearing Loss

Background: Several otologic conditions can present with fluctuating sensorineural hearing loss, including Ménière’s disease, autoimmune inner ear disease, and enlarged vestibular aqueduct. Although these 3 etiologies vary greatly, distinguishing between these conditions at initial presentation can be challenging. Furthermore, initial treatment of these conditions is often similar. In this review, we discuss historical and current perspectives on diagnosis and treatment of these conditions. Summary: A literature search was performed regarding fluctuating hearing loss, and current treatment of these etiologies of fluctuating hearing loss was summarized. Immediate measures at the onset of acute hearing loss include corticosteroid therapy, while preventative and chronic therapies, which can limit disease severity and frequency, vary based on the specific condition treated. Key Messages: Fluctuating hearing loss can represent a range of pathologies, but the precise etiology may not be clear at initial presentation. Timely treatment and long-term follow-up, along with appropriate diagnostics, are necessary to optimize long-term hearing.

Fluctuating Hearing Loss in the Only Hearing Ear: Cochlear Implantation in the Contralateral Deaf Side

Objective To investigate the hearing performance of adult patients presenting unilateral deafness with contralateral fluctuating hearing loss who received a cochlear implant on the deaf side. Study Design Case series with chart review. Setting University tertiary referral center. Subjects and Methods Preoperatively and at 6 and 12 months postoperatively, 23 patients underwent pure tone audiometry and speech audiometry with disyllabic and monosyllabic words in a quiet environment and sentences in quiet and noisy (signal-to-noise ratio +10 dB SPL) environments under best-aided conditions. The Abbreviated Profile of Hearing Aid Benefit (APHAB) inventory was evaluated preoperatively and at 6 and 12 months postoperatively. Results No difference was found between pre- and postoperative tests for disyllabic and monosyllabic words. For sentences in quiet and noisy environments, a difference between pre- and postoperative performance was present at 1 year ( P = .002 and P = .02, respectively). In a noisy environment, a difference was present at 6 and 12 months postoperatively as compared with the preoperative value (mean ± SD: 6 months: 42% ± 7.1% vs 61% ± 6.5%, P = .016). A significant improvement in the APHAB score was found at 6 and 12 months postimplantation (Friedman’s 2-way analysis of variance by ranks, P < .001). The number of years of hearing deprivation of the deaf ear was not correlated with performance. Conclusion When incapacitating fluctuating hearing loss occurs in patients presenting a contralateral deaf ear, a cochlear implant is indicated in the latter ear, significantly improving performance in noisy conditions and allowing a better quality of communication to be achieved.