Epidemic typhus fever and hearing loss: a histological study

1993 ◽  
Vol 107 (4) ◽  
pp. 275-283 ◽  
Author(s):  
I. Friedmann ◽  
A. Frohlich ◽  
A. Wright

Hearing loss as a frequent complication of louse–bome epidemic typhus fever has been well documented in the reports of ENT specialists serving in both the Allied and the German armies in the last war. The present paper describes the characteristic histopathological features as noted in sections of the temporal bones from five British soldiers who died in 1944 of typhus fever during the last war in Eastern Asia. The VHIth nerve showed multiple ‘typhus nodules’ and there was extensive interstitial neuritis of the VHIth nerve and demyelination of the nerve fibres. There were also widely scattered aggregations of mononuclear cells in the inner ear.This unique study was based on the Hallpike collection of temporal bone sections.

Author(s):  
Kiran Natarajan ◽  
Koka Madhav ◽  
A. V. Saraswathi ◽  
Mohan Kameswaran

<p>Bilateral temporal bone fractures are rare; accounting for 9% to 20% of cases of temporal bone fractures. Clinical manifestations include hearing loss, facial paralysis, CSF otorhinorrhea and dizziness. This is a case report of a patient who presented with bilateral temporal bone fractures. This is a report of a 23-yr-old male who sustained bilateral temporal bone fractures and presented 18 days later with complaints of watery discharge from left ear and nose, bilateral profound hearing loss and facial weakness on the right side. Pure tone audiometry revealed bilateral profound sensori-neural hearing loss. CT temporal bones &amp; MRI scans of brain were done to assess the extent of injuries. The patient underwent left CSF otorrhea repair, as the CSF leak was active and not responding to conservative management. One week later, the patient underwent right facial nerve decompression. The patient could not afford a cochlear implant (CI) in the right ear at the same sitting, however, implantation was advised as soon as possible because of the risk of cochlear ossification. The transcochlear approach was used to seal the CSF leak from the oval and round windows on the left side. The facial nerve was decompressed on the right side. The House-Brackmann grade improved from Grade V to grade III at last follow-up. Patients with bilateral temporal bone fractures require prompt assessment and management to decrease the risk of complications such as meningitis, permanent facial paralysis or hearing loss. </p>


1986 ◽  
Vol 95 (5) ◽  
pp. 480-486 ◽  
Author(s):  
Charles G. Wright ◽  
William L. Meyerhoff ◽  
O. E. Brown ◽  
J. C. Rutledge

CHARGE association is a recently described cluster of congenital defects including ocular coloboma, heart disease, choanal atresia, retarded development and/or CNS abnormalities, genital hypoplasia, and ear anomalies. Although congenital hearing loss has been reported in CHARGE association, no information regarding the underlying temporal bone disease is available in the literature to date. The authors evaluated four patients with multiple anomalies consistent with CHARGE syndrome. Two surviving patients have bilateral severe hearing loss on auditory brain stem response testing. Two patients did not survive, and their temporal bones were obtained at autopsy for histologic examination. All four temporal bones showed severe middle ear defects including ossicular deformities, absence of the stapedius muscle, absence of the oval window, aberrant course of the facial nerve, and dehiscence of the facial nerve canal. In the more severely affected case, a Mondini-type malformation of the cochlea was present, together with multiple anomalies of the vestibular apparatus. Vestibular defects also occurred in the other case; however, the cochleae were found to be normally developed.


2017 ◽  
Vol 131 (3) ◽  
pp. 209-214 ◽  
Author(s):  
S W Song ◽  
B C Jun ◽  
H Kim

AbstractObjective:To evaluate the clinical and radiological aspects of otic capsule sparing temporal bone fractures.Methods:Using medical records, 188 temporal bones of 173 patients with otic capsule sparing temporal bone fractures were evaluated. Otoscopic findings and symptoms, facial paralysis, and hearing loss were assessed.Results:Using regional analysis, 7 fractures were classified as type I, 85 as type II, 169 as type III and 114 as type IV. Fourteen of the 17 facial paralysis cases improved to House–Brackmann grade II or lower at an average of 57.6 days after the initial evaluation. Thirty-one patients underwent initial and follow-up pure tone audiometry examinations. The air–bone gap closed significantly from 27.2 dB at an average of 21.8 days post-trauma to 19.6 dB at an average of 79.9 days post-trauma, without the need for surgical intervention.Conclusion:Initial conservative treatment for facial paralysis or conductive hearing loss is possible in otic capsule sparing fracture cases after careful evaluation of the patient.


2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Manzoor Ahmed ◽  
Yogesh Indrasen More ◽  
Shaik Irfan Basha

We present a rare adult case of bilateral oval and round window atresia. Clinical and audiologic findings were suggestive of otosclerosis. High resolution CT Temporal bones showed unequivocal findings of bilateral oval and round window atresia. Atresia of these windows is a rare temporal bone anomaly. Presentation as an adult can confound the clinicians and warranting a closer look on the CT for atretic windows and subtle signs of otosclerosis in patients with conductive hearing loss.


2016 ◽  
Vol 126 (3) ◽  
pp. 236-240 ◽  
Author(s):  
Lilun Li ◽  
Bryan Ward ◽  
Margaret Cocks ◽  
Amir Kheradmand ◽  
Howard W. Francis

Objective: IgG4-related disease (IgG4-RD) is an idiopathic inflammatory condition that causes pseudotumor formation in single or multiple organs, including those of the head and neck. Temporal bone involvement is rare, with only 3 cases of unilateral temporal bone IgG4-RD described in the literature. We report the first known case of IgG4-RD of bilateral temporal bones and describe its clinical presentation, diagnosis, and treatment. Methods: The patient was a 52-year-old man with latent tuberculosis (TB) who presented with a 10-year history of bilateral profound hearing loss and vestibular dysfunction. Computed tomography and magnetic resonance imaging demonstrated bilateral labyrinthine destruction with invasion of the posterior fossa. Results: Immunoglobulin level testing showed elevated total serum IgG levels with normal IgG4 levels. Bilateral mastoidectomies were performed, with biopsy samples demonstrating IgG4 staining with IgG4-positive plasma cells up to 40/HPF (high power field) on the right and 20/HPF on the left, consistent with bilateral IgG4-RD. Conclusion: IgG4-RD of bilateral temporal bones presents with chronic and progressive bilateral hearing loss and vestibular dysfunction. Clinical presentation and radiologic findings are nonspecific, and definitive diagnosis must be made with histopathology and immunostaining. Corticosteroids are therapeutic, but surgical resection may be necessary for temporal bone IgG4-RD to improve long-term remission.


2008 ◽  
Vol 123 (6) ◽  
pp. 676-679 ◽  
Author(s):  
J K Zlodre ◽  
A T M Rennie ◽  
J D Ramsden

AbstractObjective:To present the successful treatment of sensorineural hearing loss secondary to Langerhans' cell histiocytosis with low-dose radiotherapy, and also the disparity between radiological resolution of Langerhans' cell histiocytosis lesions and lack of sensorineural hearing loss improvement, accompanied by a review of the literature on otolaryngological manifestations and management of Langerhans' cell histiocytosis.Case report:Langerhans' cell histiocytosis is a multisystem disease which frequently causes osseous lesions in the temporal bones. Hearing loss is usually conductive but may be sensorineural with lesions of the petrous temporal bone. We present a case of sensorineural hearing loss secondary to Langerhans' cell histiocytosis affecting the labyrinth and internal auditory meatus, which resolved following radiotherapy. Contralateral sensorineural hearing loss in the same patient, previously treated with chemotherapy, did not resolve despite radiological resolution of the temporal bone lesions.Conclusion:We suggest that timely radiotherapy for treatment of sensorineural hearing loss secondary to Langerhans' cell histiocytosis is an appropriate treatment option.


1980 ◽  
Vol 89 (3) ◽  
pp. 251-252 ◽  
Author(s):  
Joseph B. Nadol

Using a specifically designed circular bone plug cutter, an extracranial technique to obtain temporal bones for histological study is described. This technique is useful in cases in which conventional autopsy of the head it not possible or desirable.


2010 ◽  
Vol 3 (2) ◽  
pp. 105-113 ◽  
Author(s):  
Alpen Patel ◽  
Eli Groppo

The temporal bones are paired structures located on the lateral aspects of the skull and contribute to the skull base. Trauma is usually the result of blunt head injury and can result in damage to the brain and meninges, the middle and internal ear, and the facial nerve. Complications can include intracranial hemorrhage, cerebral contusion, CSF leak and meningitis, hearing loss, vertigo, and facial paralysis. To prevent these complications, diagnosis followed by appropriate medical and surgical management is critical. Diagnosis relies primarily on physical signs and symptoms as well as radiographic imaging. Emergent intervention is required in situations involving herniation of the brain into the middle ear cavity or hemorrhage of the intratemporal carotid artery. Patients with declining facial nerve function are candidates for early surgical intervention. Conductive hearing loss can be corrected surgically as an elective procedure, while sensorineural hearing loss carries a poor prognosis, regardless of management approach. Children generally recover from temporal bone trauma with fewer complications than adults and experience a markedly lower incidence of facial nerve paralysis.


2018 ◽  
Vol 22 (4) ◽  
pp. 25-30
Author(s):  
Sergey A. Ivanov ◽  
Sergey G. Zhuravskii

Paget’s disease of bone is a localized disorder of bone remodeling. These functional and structural alterations, interacting with the specific characteristics of the site of involvement, account for most of the complications of the disease. Bilateral progressive hearing loss is the most frequently encountered complication of Paget’s disease, because of the involvement of the temporal bones. There may be difficulty in distinguishing patients with presbycusis from those with Paget’s disease-related hearing loss. Pagetic hearing loss seems to be stabilized by effective medical treatment. For patients with severe hearing loss, cochlear implantation may be recommended.


2021 ◽  
Vol 15 ◽  
Author(s):  
Nicholas S. Andresen ◽  
Sarah Coreas ◽  
Dillan F. Villavisanis ◽  
Amanda M. Lauer

BackgroundMelanin pigmentation is present within the auditory and vestibular systems of the mammalian inner ear and may play a role in maintaining auditory and vestibular function. Melanocytes within the stria vascularis (SV) are necessary for the generation of the endocochlear potential (EP) and decreased EP has been linked to age-related hearing loss. Melanocytes and pigment-containing “dark cells” are present within the vestibular system, but have a less well-defined role. African–American individuals have increased pigmentation within the SV and vestibular system, which is hypothesized to be related to lower rates of age-related hearing loss and vestibular dysfunction. It remains unclear if increased pigmentation confers lifelong protection against hearing loss and vestibular dysfunction.MethodsMouse temporal bones were collected from juvenile (3–4 week) and aged (20–32 months) CBA/CaJ mice. Pediatric and adult human temporal bones from Caucasian or African–American individuals were examined from the Johns Hopkins Temporal Bone Collection. Information regarding Fitzpatrick skin type were unavailable, and self-identified race/ethnicity was used as a proxy. Images were taken using light microscopy at 20× magnification. ImageJ software (v1.53) was used to measure pigment within the SV and vestibular system.ResultsIn mouse temporal bones pigmentation within the SV increased with age, but pigmentation within the vestibular system did not increase with age. In human temporal bones pigmentation within the SV increased with age and pigmentation within the vestibular system increased within the wall of the utricle, but not other regions of the vestibular system. African–American individuals had higher amounts of pigment within the SV and vestibular system, among both pediatric and adult populations.ConclusionStria vascularis pigmentation increases with age in mouse and human temporal bones. Pigmentation within the vestibular system did not increase with age in mouse specimens and only increased within the utricular wall with age in human specimens. Individuals who identified as African–American had higher pigment content within the SV and vestibular system, both as children and as adults. These results highlight how similar age-related pigmentary changes occur in the auditory and vestibular systems across species and underscore the importance of racial/ethnic diversity in human temporal bone studies.


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