Ataxia and Hearing Loss Secondary to Perilymphatic Fistula

PEDIATRICS ◽  
1978 ◽  
Vol 61 (2) ◽  
pp. 238-241
Author(s):  
Gerald B. Healy ◽  
Jules M. Friedman ◽  
Joseph DiTroia
Keyword(s):  
Hearing Loss ◽  
Soft Tissue ◽  
Head Trauma ◽  
Middle Ear ◽  
Vestibular System ◽  
Perilymphatic Fistula ◽  
Labyrinthine Fistula ◽  
First Case ◽  
The Absolute ◽  
Physical Findings

Ataxia is rarely attributed to lesions of the peripheral vestibular system. In 1973, the first case of ataxia and hearing loss secondary to a labyrinthine fistula was reported. Until now, this syndrome has not been reported in patients under the age of 10 years. A case is presented of a 5-year-old boy with symptoms of ataxia and hearing loss as well as vertigo and tinnitus after head trauma. Three physical findings appear to be most characterisitic of patients with perilymphatic fistulas: a positive fistula response, positive positional testing with the involved ear down, and evidence of vestibular ataxia when testing station and gait. The absolute diagnosis of perilymphatic fistula can only be established by exploration of the middle ear space. If a fistula is found, it may be sealed with soft tissue and, if this fails, actual stapedectomy may be required.

scholarly journals Unusual presentation of tubercular mastoid cyst

2021 ◽  
Vol 9 ◽  
pp. 2050313X2110564
Author(s):  
Neeraj Rauniyar ◽  
Dipesh Shakya
Keyword(s):  
Hearing Loss ◽  
Otitis Media ◽  
Middle Ear ◽  
Chronic Otitis Media ◽  
Signs And Symptoms ◽  
Diagnostic Dilemma ◽  
First Case ◽  
Tuberculous Otitis Media ◽  
High Index Of Suspicion ◽  
Tuberculous Mastoiditis

Tuberculosis in the otomastoid compartment is extremely rare. The classical presentation of tuberculous otitis media includes chronic otorrhea, severe hearing loss irrespective of the disease, multiple perforations, and pale granulations in the middle ear cavity. However, most present with non-specific signs and symptoms making it a diagnostic dilemma. Here, we report a case that presented with a bony mastoid cyst which is the first case report to the best of our knowledge. A 12-year-old boy visited our center with a complaint of chronic ear discharge and hearing loss not improving with conventional antibiotic therapy. The computed tomography scan of the temporal bone revealed soft tissue opacification in the mastoid along with features suggestive of cholesteatomatous chronic otitis media. The patient underwent mastoidectomy under general anesthesia, which revealed a bony mastoid cyst filled with turbid fluid along with granulation tissues in the middle ear cleft without cholesteatoma. Final histopathology revealed tuberculous otitis media. Antitubercular chemotherapy was started and the patient’s condition recovered. Primary tuberculous mastoiditis is a rare clinical entity that requires a high index of suspicion. It can also present as chronic otorrhea with mastoid cyst, and thus need to be considered as one of the differential diagnoses.

Perilymphatic Fistula: A Histopathologic Study

1986 ◽  
Vol 95 (5) ◽  
pp. 466-471 ◽  
Author(s):  
Robert I. Kohut ◽  
Raul Hinojosa ◽  
Joseph A. Budetti
Keyword(s):  
Middle Ear ◽  
Clinical History ◽  
Histologic Diagnosis ◽  
Perilymphatic Fistula ◽  
Clinical Criteria ◽  
Histopathologic Study ◽  
Temporal Bones ◽  
The University ◽  
Physical Findings ◽  
Chicago Medical

Over the last two decades, clinical criteria for perilymphatic fistulae have been defined to the extent that differentiation can be made between such fistulae and other balance-affecting disorders such as Meniere's syndrome. On the assumption that the specimens in the temporal bone bank of the University of Chicago Medical School that had been obtained from patients having vertigo, hearing loss, or both, before those clinical criteria were so defined might have been classified incorrectly, we proposed a retrospective histopathologic study, with prediction of two independent variables: 1) a clinical history and physical findings consistent with the diagnosis of perilymphatic fistula and 2) communication between the vestibule and the middle ear adjacent to or via the fissula ante fenestram. Eleven pairs of temporal bones with the histologic diagnosis of idiopathic labyrinthine hydrops were evaluated before the clinical histories relevant to those specimens were reviewed. In one specimen, a communication between the vestibule and the middle ear space was identified. In none of the other specimens was there a similar communication. As this study continued, significance was given to the histologic details of the communication between the middle ear and posterior canal ampulla. The temporal bones without these communications did not have clinical histories consistent with the diagnosis of perilymphatic fistula.

scholarly journals Complete Restored Hearing Loss after Excessive Nose Blowing with Various CT Findings

2020 ◽  
Vol 63 (12) ◽  
pp. 620-624
Author(s):  
Ji Hoon Koh ◽  
Eun Jung Lee
Keyword(s):  
Hearing Loss ◽  
Temporal Bone ◽  
Middle Ear ◽  
Bone Fracture ◽  
Valsalva Maneuver ◽  
Pressure Change ◽  
Temporal Bone Fracture ◽  
Perilymphatic Fistula ◽  
Ct Findings ◽  
Air Pockets

A perilymphatic fistula (PLF) is defined as leakage of perilymph with several possible causes such as superior canal dehiscence through trauma, temporal bone fracture, or sudden pressure change (e.g., skydiving or scuba diving). Pneumolabyrinth can result from temporal bone fracture after trauma, or sudden pressure change in the middle ear or cerebrospinal fluid, such as excessive nose blowing or Valsalva maneuver. A PLF and pneumolabyrinth may occur without trauma, associated with a sudden pressure change in the middle ear. We report two cases of PLF followed by pneumolabyrinth and one case of suspicious PLF without pneumolabyrinth after excessive nose blowing. All three cases were diagnosed as having sensorineural hearing loss and the patients recovered completely after conservative treatment. We report various CT findings of pneumolabyrinth and PLF, from normal CT findings to air pockets in the labyrinth and soft tissue density around the stapes.

scholarly journals Isolated Incudostapedial Cholesteatomas: Unique Radiologic and Surgical Features

2020 ◽  
pp. 014556132097378
Author(s):  
Bridget MacDonald ◽  
Krishna Bommakanti ◽  
Moises Mallo ◽  
Daniela Carvalho
Keyword(s):  
Hearing Loss ◽  
Soft Tissue ◽  
Ct Scan ◽  
Middle Ear ◽  
Conductive Hearing Loss ◽  
Soft Tissue Mass ◽  
Ossicular Chain ◽  
Ct Scans ◽  
Tissue Mass ◽  
Conductive Hearing

Objectives: Congenital cholesteatomas originate from epithelial tissue present within the middle ear in patients with an intact tympanic membrane, no history of otologic surgery, otorrhea, or tympanic membrane perforation. They are diagnosed by a pearl-like lesion on otoscopy and computed tomography (CT) scan showing an expansile soft-tissue mass. We describe a series of patients with no prior otologic history presenting with progressive unilateral conductive hearing loss and normal otoscopy. The CT scans showed ossicular erosion without obvious soft-tissue mass. Surgery confirmed incudostapedial erosion found to be cholesteatoma. In this study, we characterize the clinical course of patients diagnosed with isolated incudostapedial cholesteatoma (IIC) and review possible pathologic mechanisms. Methods: Retrospective review of IIC cases treated by the Department of Pediatric Otolaryngology, Rady Children’s Hospital, San Diego, 2014 to 2020. Data included patient demographics, clinical features, imaging, surgical findings, and audiologic data. Results: Five patients were diagnosed with IIC (3 [60%] female; mean age at presentation 10.7 years [range 5.5-16.0]). All patients presented with postlingual unilateral conductive hearing loss and normal otoscopy without any past otologic history; delay in diagnosis ranged from 4 months to several years. The CT scans showed ossicular chain erosion with an absent long process of the incus and/or stapes superstructure. All patients underwent middle ear exploration, revealing a thin layer of cholesteatoma in the incudostapedial region, confirmed by histopathology. Mean preoperative speech reception threshold was 55 dB and improved to a mean of 31 dB in the 4 patients who underwent ossicular chain reconstruction. Conclusion: Isolated incudostapedial cholesteatoma should be included as a possible etiology in pediatric patients with insidious onset of unilateral conductive hearing loss with normal otoscopy, unremarkable otologic history, and a CT scan showing ossicular abnormality/disruption without notable middle ear mass. These patients should be counseled preoperatively regarding the possibility of cholesteatoma and should undergo middle ear exploration with possible ossiculoplasty.

Primary synovial sarcoma of the middle ear

1993 ◽  
Vol 107 (11) ◽  
pp. 1070-1072 ◽  
Author(s):  
L. J. O'keei ◽  
R. T. Ramsden ◽  
A. R. Birzgalis
Keyword(s):  
Young Adults ◽  
Soft Tissue ◽  
Head And Neck ◽  
Synovial Sarcoma ◽  
Synovial Tissue ◽  
Middle Ear ◽  
Neck Region ◽  
Lower Limbs ◽  
Head And Neck Region ◽  
First Case

AbstractSynovial sarcoma is a soft tissue malignancy which most commonly affects the lower limbs of young adults and rarely occurs in the head and neck region. The term synovial sarcoma may be a misnomer as most of these tumours occur in tissues not known to contain synovial tissue. There has only been one previously reported case affecting the middle ear, which was metastatic, and we report the first case of primary synovial sarcoma of the middle ear.

Air Conduction, Bone Conduction, and Soft Tissue Conduction Audiograms in Normal Hearing and Simulated Hearing Losses

2015 ◽  
Vol 26 (01) ◽  
pp. 101-108 ◽  
Author(s):  
Cahtia Adelman ◽  
Adi Cohen ◽  
Adi Regev-Cohen ◽  
Shai Chordekar ◽  
Rachel Fraenkel ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Differential Diagnosis ◽  
Soft Tissue ◽  
Middle Ear ◽  
Bone Conduction ◽  
Auditory Stimulation ◽  
Threshold Shift ◽  
N Application ◽  
Similar Threshold ◽  
Air Conduction

Background: In order to differentiate between a conductive hearing loss (CHL) and a sensorineural hearing loss (SNHL) in the hearing-impaired individual, we compared thresholds to air conduction (AC) and bone conduction (BC) auditory stimulation. The presence of a gap between these thresholds (an air-bone gap) is taken as a sign of a CHL, whereas similar threshold elevations reflect an SNHL. This is based on the assumption that BC stimulation directly excites the inner ear, bypassing the middle ear. However, several of the classic mechanisms of BC stimulation such as ossicular chain inertia and the occlusion effect involve middle ear structures. An additional mode of auditory stimulation, called soft tissue conduction (STC; also called nonosseous BC) has been demonstrated, in which the clinical bone vibrator elicits hearing when it is applied to soft tissue sites on the head, neck, and thorax. Purpose: The purpose of this study was to assess the relative contributions of threshold determinations to stimulation by STC, in addition to AC and osseous BC, to the differential diagnosis between a CHL and an SNHL. Research Design: Baseline auditory thresholds were determined in normal participants to AC (supra-aural earphones), BC (B71 bone vibrator at the mastoid, with 5 N application force), and STC (B71 bone vibrator) to the submental area and to the submandibular triangle with 5 N application force) stimulation in response to 0.5, 1.0, 2.0, and 4.0 kHz tones. A CHL was then simulated in the participants by means of an ear plug. Separately, an SNHL was simulated in these participants with 30 dB effective masking. Study sample: Study sample consisted of 10 normal-hearing participants (4 males; 6 females, aged 20–30 yr). Data Collection and Analysis: AC, BC, and STC thresholds were determined in the initial normal state and in the presence of each of the simulations. Results: The earplug-induced CHL simulation led to a mean AC threshold elevation of 21–37 dB (depending on frequency), but not of BC and STC thresholds. The masking-induced SNHL led to a mean elevation of AC, BC, and STC thresholds (23–36 dB, depending on frequency). In each type of simulation, the BC threshold shift was similar to that of the STC threshold shift. Conclusions: These results, which show a similar threshold shift for STC and for BC as a result of these simulations, together with additional clinical and laboratory findings, provide evidence that BC thresholds likely represent the threshold of the nonosseous BC (STC) component of multicomponent BC at the BC stimulation site, and thereby succeed in clinical practice to contribute to the differential diagnosis. This also provides evidence that STC (nonosseous BC) stimulation at low intensities probably does not involve components of the middle ear, represents true cochlear function, and therefore can also contribute to a differential diagnosis (e.g., in situations where the clinical bone vibrator cannot be applied to the mastoid or forehead with a 5 N force, such as in severe skull fracture).

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