Bilateral Sudden Sensorineural Hearing Loss and Intralabyrinthine Hemorrhage in a Patient With COVID-19.

OBJECTIVE: To describe a case of bilateral sudden sensorineural hearing loss (SSNHL) and intralabyrinthine hemorrhage in a patient with COVID-19. STUDY DESIGN: Clinical capsule report. SETTING: Tertiary academic referral center. PATIENT: An adult woman with bilateral SSNHL, aural fullness, and vertigo with documented SARS-CoV-2 infection (IgG serology testing). INTERVENTIONS: High-dose oral prednisone with taper, intratympanic dexamethasone. MAIN OUTCOME MEASURES: Audiometric testing, MRI of the internal auditory canal with and without contrast. RESULTS: A patient presented with bilateral SSNHL, bilateral aural fullness, and vertigo. Serology testing performed several weeks after onset of symptoms was positive for IgG COVID-19 antibodies. MRI showed bilateral intralabyrinthine hemorrhage (left worse than right) and no tumor. The patient was treated with two courses of high-dose oral prednisone with taper and a left intratympanic dexamethasone injection, resulting in near-resolution of vestibular symptoms, a fluctuating sensorineural hearing loss in the right ear, and a severe to profound mixed hearing loss in the left ear. CONCLUSIONS: COVID-19 may have otologic manifestations including sudden SSNHL, aural fullness, vertigo, and intralabyrinthine hemorrhage.

[Viral infection and ear diseases].

The association of viral infection to ear disease has triggered a great deal of interests. In the present paper, we provide a critical review of the viral hypothesis of ear diseases. Detection of viral antigen and antibody or RNA and DNA in the patients serum, endolymphatic fluid or surgical pathology specimens reveals that virus may have relevance to certain kinds of ear diseases, such as Meniere's disease, idiopathic sudden sensorineural hearing loss, otosclerosis. Bell's palsy and otitis media. The most appealing is the herpesvirus, which can cause latent infection in the neurons, and its reactivation may be the mechanism of recurrent attacks of ear diseases. Currently, antiviral drug treatment plus supportive therapy are the most effective managements dealing with viral infection. Although antiviral vaccine will become a promising preventive strategy in the future.

Inner ear lesions in congenital cytomegalovirus infection of human fetuses.

Congenital cytomegalovirus (CMV) infection is the leading cause of non-hereditary congenital sensorineural hearing loss (SNHL). The natural course and the pathophysiology of inner ear lesions during human fetal CMV infection have not yet been reported. Inner ear lesions were investigated in six CMV-infected fetuses aged 19-35 postconceptional weeks and correlated with central nervous system (CNS) lesions. All the fetuses had high viral loads in the amniotic fluid and severe visceral and CNS lesions visible by ultrasound. Diffuse lesions consisting of both cytomegalic cells containing inclusion bodies and inflammation were found within all studied structures including the inner ear, brain, other organs, and placenta, suggesting hematogenous dissemination. Cochlear infection was consistently present and predominated in the stria vascularis (5/6), whereas the supporting cells in the organ of Corti were less often involved (2/6). Vestibular infection, found in 4/6 cases, was florid; the non-sensory epithelia, including the dark cells, were extensively infected. The endolymphatic sac was infected in 1 of 3 cases. The severity of inner ear infection was correlated with the CNS lesions, confirming the neurotropism of CMV. This study documenting infection of the structures involved in endolymph secretion and potassium homeostasis in fetuses with high amniotic fluid viral loads suggests that potassium dysregulation in the endolymphatic compartment of the inner ear may lead to secondary degeneration of the sensory structures. In addition, the occurrence of SNHL depends on the intensity and duration of the viral infection and inflammation.

Murine model for congenital CMV infection and hearing impairment.

BACKGROUND: Congenital cytomegalovirus (CMV) infection is the leading cause of sensorineural hearing loss (SNHL), and SNHL is the most frequent sequela of congenital CMV infection. But the pathogenic mechanism remains unknown, and there is no ideal CMV intrauterine infection animal model to study the mechanisms by which SNHL develops. METHODS: We established the congenital murine cytomegalovirus (MCMV) infection model by directly injecting the virus into the placenta on day 12.5 of gestation. Then, we observed the development and the MCMV congenital infection rate of the fetuses on the day they were born. Furthermore, we detected the auditory functions, the conditions of the MCMV infection, and the histological change of the inner ears of 28-day-old and 70-day-old offspring. RESULTS: Both the fetal loss rate and the teratism rate of offspring whose placentas were inoculated with MCMV increased, and their body length, head circumference, and weight decreased. The hearing level of offspring both decreased at both 28- and 70-days post birth; the 70-day-old mice developed lower hearing levels than did the 28-day old mice. No significant inflammatory changes in the cochleae of the mice were observed. MCMV DNA signals were mainly detected in the spiral ganglion neurons and the endolymph area, but not in the perilymph area. The number of neurons decreased, and their ultrastructures changed. Moreover, with age, the number of neurons dramatically decreased, and the ultrastructural lesions of neurons became much more severe. CONCLUSIONS: The results suggest that the direct injection of MCMV into the placenta may efficiently cause fetal infection and disturb the intrauterine development of the fetus, and placental inoculation itself has no obvious adverse effects on offspring. The reduction in the number of spiral ganglion neurons and the ultrastructural lesions of the neurons may be the major cause of congenital CMV infection-induced progressive SNHL.

[A case of herpetic facial paralysis in which cochleovestibular symptoms outweigh facial nerve symptoms]. / Kokleovestibüler semptomlarin ön planda oldugu herpetik fasyal paralizi: Olgu sunumu.

A 42-year-old man presented with sensorineural hearing loss of acute onset, tinnitus, and vertigo. Physical examination revealed slight asymmetry in facial nerve functions and spontaneous nystagmus. Magnetic resonance imaging of the internal acoustic canal showed contrast enhancement consistent with edema-inflammation, being notable and diffuse in the seventh and eighth cranial nerve complex, and minimal in the cochlea. Non-hydropic cochleovestibular syndrome was considered and the patient was treated with antiviral and corticosteroid medications. A week later, facial paralysis improved and the acute hearing loss reversed. On the twelfth day of presentation, he had no complaints other than mild imbalance on abrupt changes in movement. In this type of herpetic facial paralysis in which cochleovestibular symptoms outweigh facial nerve symptoms, it might be argued that varicella zoster virus reactivation occurs in the spiral and/or vestibular ganglion.

Does the serological study for viral infection in autoimmune inner ear disease make sense?

Viral infections of the labyrinth have been considered a major source of auditory and vestibular system pathology. However, the involvement of virus in the development of immune reactions responsible for immunomediated inner ear disease has not been studied enough. Following viral infection, an effector immune response, humoral (B cell) and/or cytotoxic (T cell) is directed against a virus and it might cross-react with self-protein or autoantigen, evoking an autoimmune response. Since clinically it can be very difficult to establish a viral etiology for such disorders, serologic studies can be used to confirm the suspected diagnosis. Patients affected by immunomediated inner ear disease that had presented an upper respiratory tract infection underwent an immunologic workup study including microbiological study. After the application of this diagnostic protocol, only one patient, that was subsequently diagnosed with Cogan's syndrome, showed a positive serological test for viral infection. On the basis of the low efficacy of serological testing and due to the lack of evidence, we do not recommend to carry out serologic studies for viral infection.

Do viruses cause inner ear disturbances?

The association of viral infection to inner ear disease is controversial. Experiments on animals show that several viruses are capable of causing hearing loss, if applied into the perilymph. Some of these have specific affinity to the cellular type of the inner ear, as sensory epithelia and cochlear nerve. Some viruses as adenoviruses and Coxsackie virus B have specific CAR receptors that are identified in different cell types, whereas other act by attaching onto nonspecific cellular surface receptors. Some viruses such as varicella zoster virus (VZV) do not cause disease in rodents. We assessed 273 patients with clinical, serological, neuro-otologic and endoscopic evaluations. Of the 273 patients, 43 served as control subjects. The patients either had Ménière's disease (n = 158), recurrent vertigo of unknown etiology (n = 56), or hearing loss (n = 17). Antibodies against neurotropic and common viruses were evaluated. VZV, influenza B, CBV5 and RSV titers were significantly elevated in patients with inner ear disease when compared with the control group. In analyzing the internal relationship, VZV and influenza B were intercorrelated. We did not find a correlation between hearing loss and viral titers. In conclusion, VZV, Coxsackie virus B5 and influenza B virus may be the main causes of inner ear disorder. The spiral and Scarpa's ganglion are potential sites harboring viral DNA for possible latent infection.

Viral etiology for inner ear diseases: proven, unproven, unlikely.

This is a revision article that deals with the broad field of inner ear disease caused by viral infections. Some of these entities have been proven to have a viral etiology. Others have strong evidence in favor of a viral causation but still cannot be considered as a viral disease. Finally, other entities are suggestive of a viral etiology but when the whole body of evidence is considered one concludes that a viral etiology is indeed unlikely. We review the literature and add our own experience in this subject. Clearly, the most important evidence about this subject came from the study of temporal bone histopathology. Certainly, we can learn much more if we continue to collect and study temporal bone specimens histopathologically.

Clinical viral infections and temporal bone histologic studies of patients with AIDS.

The brain, eye, and inner ear are each protected from blood-borne infectious agents by a barrier that has some anatomic and functional differences. In patients with AIDS, opportunistic infections of the central nervous system and eye are frequent. Little is known about the incidence of middle and inner ear infections in patients with AIDS, but deafness and severe vertigo are uncommon. We studied 14 homosexual men with AIDS, aged 28 to 55 years, for 1 to 2 years until death. No patient had deafness, but one had vertigo. Adenovirus type 6 and cytomegalovirus were isolated from the middle ear cavity in four patients. Temporal bone histology demonstrated acute otitis media in four, chronic otitis media in two, and serous otitis media in three. Adenovirus type 6 and cytomegalovirus, either alone or with herpes simplex virus type 1, were isolated from inner fluids of three patients. Histologic inner ear findings were abnormal in only one patient. Viruses were isolated or histologically identified in the brains of four patients and in the eyes of five patients. In our patients viral infections were nearly as common in the inner ears as in the brain and eye, suggesting that protection from the blood-labyrinth barrier was similar to that from the other barriers. Because the inner ear viral infections were asymptomatic and there was an absence of pathologic damage and inflammation, we suggest that some viral inner ear infections in patients with AIDS are nonpathogenic and elicit no inflammation or that the viral infections occur terminally and elicit no inflammation because of immunosuppression from the AIDS.

Immune-mediated inner ear disease and parvovirus B19.

Human parvovirus B19 (HP-B19), the etiologic agent of the common childhood illness erythema infectiosum, has been implicated in systemic immune disorders. Patients presenting with sensorineural hearing loss and/or dizziness, not readily categorized, were evaluated for immune-mediated inner ear disease. Appropriate serologic studies including parvovirus B19 antibody titers were conducted. Thirty patients with suspected immune-mediated inner ear disease were treated with corticosteroid trial for 5 to 7 days, then reexamined with repeat audiogram and/or vestibular testing. Seventeen patients with clinical response were treated with long-term corticosteroids and cyclophosphamide. Six patients had positive HP-B19 immunoglobulin M (IgM) titers and 8 patients had positive parvovirus immunoglobulin G (IgG) titers. All 14 patients responded to therapy. Parvovirus B19, therefore, is a possible etiology of immune-mediated inner ear disease.