Excessive and persistent noise damages the nerve endings present in the inner ear which can lead to permanent hearing loss. Nerve damage in the ear can also cause tinnitus, which is a condition where the patient feels persistent ringing in the ears. Brain tumors or tumors of the skull can also cause damage to the vestibular or acoustic branches of the vestibulocochlear nerve.
Acoustic neuroma is a noncancerous growth thought to occur as a result of genetic factors. This tumor can develop in vestibular portion of the nerve and is also termed as vestibular schwannoma. Some of the common symptoms of Acoustic neuroma include balance disorders , hearing loss and tinnitus. Treatment depends on the cause of the nerve damage and can include the use of medications, surgery and hearing aids.
Although shingles looks like a bad skin rash — and it is — the rash is actually a manifestation of a central nervous system disease. That explains why, when shingles emerges in the auditory system, it almost always affects only one ear — the one surrounded by the rash, as shown in the feature image. Besides intense and severe pain in and around the ear, other symptoms can occur, such as:. When the varicella zoster virus reactivates into Shingles, it has a choice of nerve paths to follow.
The American Academy of Otolaryngology-Head and Neck Surgery recommends the following when beginning to wear hearing aids: Be patient and give yourself time to get used to the hearing aid and the sound it produces. Start in quiet surroundings and gradually build up to noisier environments. Experiment where and when the hearing aid works best for you.
Taking care of hearing aids Hearing aids need to be kept dry. Other tips for taking care of hearing aids include: Keep the hearing aids away from heat and moisture. Batteries should be replaced on a regular basis. Avoid the use of hairspray and other hair products when the hearing aid is in place. Turn off hearing aids when they are not in use.
Considerations when purchasing a hearing aid A medical examination is required before purchasing a hearing aid. The National Institute on Deafness and Other Communication Disorders recommends asking the following questions when buying hearing aids: Can the hearing loss be improved with medical or surgical interventions?
Which design will work best for my type of hearing loss? May I "test" the hearing aids for a certain period? How much do hearing aids cost? Do the hearing aids have a warranty and does it cover maintenance and repairs? Can my audiologist or otolaryngologist make adjustments and repairs? Congenital rubella syndrome has not been reported following birth from mothers who were asymptomatically infected during pregnancy.
Due to successful vaccination programs, rubella is currently considered eliminated in the United States; however, cases can still occur due to importation of the infection from other countries McLean et al. Infection is transmitted to humans through contact with rodent urine, feces, or saliva, and occurs more commonly in winter months when mice seek shelter indoors Bonthius, The virus is not typically spread between humans; however, there have been cases of transmission via organ transplantation Jamieson et al.
In immunocompetent adults, LCMV infection is typically either asymptomatic or associated with upper respiratory tract infection symptoms fever, headache, nausea, and vomiting. Rarely, complications such as aseptic meningitis and meningoencephalitis occur. LCMV infection in pregnancy greatly increases the risk of spontaneous abortion. LCMV infection can also be teratogenic, especially if the virus is contracted during the first or second trimester, and is associated with microcephaly, hydrocephalus, ventriculomegaly, pachygyra, cerebellar hypoplasia, chorioretinitis, periventricular calcification, and hearing loss Anderson et al.
In contrast to congenital CMV or rubella, visual impairment and microcephaly are much more common than hearing loss in congenital LCMV infection.
LCMV can also be distinguished from these other congenital viral causes of hearing loss by the lack of hepatosplenomegaly Barton et al.
Fetal 28 weeks gestation a and postnatal week of life 1 b MRI of infant with congenital LCMV infection, demonstrating severe ventriculomegaly asterisks. The patient had severe growth and developmental delays, mild myopia with chorioretinopathy, profound hearing loss on the left side, and severe loss of hearing on the right. Source: MRI images used with permission from Anderson et al.
Hearing loss in these patients is relatively rare, can vary in severity between ears, and ranges from severe to profound SNHL Anderson et al. However, ribavirin efficacy against LCMV has not been proven in clinical trials and is associated with significant side effects such as hemolytic anemia.
Ribavirin is a teratogen in many animal models and should not be used to treat pregnant women Jamieson et al. Treatment of hearing loss in affected children with hearing aids and other assistive listening devices is indicated when appropriate. Treatment of severe to profound SNHL in children with congenital LCMV may be limited in patients in whom involvement of the vestibulocochlear nerve is the cause of hearing loss; however, because severe visual impairment is seen in all children with congenital LCMV infection, it should be attempted.
Symptoms of the initial infection are nonspecific and include fever, headache, sore throat, and myalgias. As the disease progresses and immune deficiency develops, patients develop opportunistic infections and other manifestations of HIV in multiple organ systems.
Common symptoms within the temporal bone include hearing loss, tinnitus, chronic otitis media, facial nerve palsy, and malignancies Palacios et al. Audiograms performed in a U. Infants can develop hearing loss following either infection or exposure in utero without infection Torre et al.
Hearing loss associated with HIV infection can be unilateral or bilateral, progressive or sudden, and conductive, sensorineural, or mixed. Hearing loss in HIV-infected patients can be caused by a number of factors, including the direct effects of HIV, increased susceptibility to opportunistic infections in the middle ear and brain, and treatment with potentially ototoxic medications Khoza-Shangase et al.
CHL often results from recurrent otitis media, otitis externa, acquired aural atresia, cholesteatoma, formation of aural polyps, or malignancy Rarey, SNHL is more common in adults and can have a variety of causes, including direct damage to the auditory system by HIV, opportunistic infections, and ototoxicity.
Typically, SNHLs are mild to moderate and predominantly include high frequencies Chandrasekhar et al. Severity of hearing loss has been correlated with disease progression and decline in CD4 counts van der Westhuizen et al.
This might lead some to suspect that hearing loss in patients with HIV might be prevented by antiretroviral therapy.
A variety of studies have demonstrated that HIV may affect the auditory system both centrally and peripherally. Because HIV-positive patients often present with multiple risk factors for hearing loss, it can be impossible to ethically determine the exact etiology of the observed hearing losses. Thus, development of an animal model for these studies would significantly improve understanding of the direct role of HIV in hearing loss.
HIV prevention is predominantly guided by avoidance of contact with infected blood and bodily secretions. HAART use does not significantly reverse hearing loss, and one study suggested a positive correlation between hearing loss and use of antiretroviral medications in patients older than 35 years Marra et al.
Following cochlear implantation, these patients should be closely followed to avoid development of chronic otitis media and its related complications Vincenti et al. HSV types 1 and 2 have been implicated as causes of hearing loss.
Both are encapsulated, double-stranded DNA viruses of the herpesvirus family. Infection follows contact of mucous membranes or broken skin surfaces with infected fluids from herpes sores or with other body fluids of patients with herpes. The initial infection may be asymptomatic or can be associated with symptoms. For HSV1, these symptoms can include painful blisters on the lips and tongue that eventually rupture, dysphagia, fever, myalgias, and sore throat. In the case of HSV2 primary infection, symptoms include tingling in the affected areas, followed by an erythematous papular rash that evolves into blisters and then ruptured open lesions, as well as a viral prodrome and headache.
The viruses can latently infect nerve cells innervating the initially infected tissue. Months to years later, the viruses can reactivate, leading to recurrent disease. Neonatal infection is more frequent from women who develop infection late during pregnancy or who have active herpetic lesions in the birth canal.
Sequelae of neonatal infection range from eye and mucous membrane involvement to disseminated disease, encephalitis, hearing loss, mental retardation, microcephaly, and death. Many infected infants will not have a vesicular rash and so may not be tested for HSV infection reviewed in Westerberg et al.
However, hearing loss following HSV1 infection is relatively rare and typically associated with concomitant severe neurological complications.
Hearing loss following neonatal infection can be bilateral or unilateral severe to profound SNHL Westerberg et al. HSV1 infection has also been associated with hearing loss following infection or reactivation of latent infection after infancy. Some of these cases are associated with herpes simplex meningitis or encephalitis.
In one case, a 3-year-old boy suddenly developed a profound bilateral hearing loss following a mild febrile illness. In another report, a year-old woman who developed oral herpes lesions and subsequent bilateral profound hearing loss had no recovery of hearing following treatment with prednisone and acyclovir.
Her serological testing was consistent with HSV1 reactivation Rabinstein et al. Animal studies have confirmed that herpes simplex infections can cause hearing loss and vestibular symptoms. Following infection with HSV1 or HSV2, fibrosis of the scala tympani and vestibule, loss of outer hair cells, and atrophy of the stria vascularis and tectorial membrane were found in these animals.
Viral antigens were located throughout the cochlea, and viral capsids were found within cochlear nerve fibers, including both afferent and efferent nerve endings. Prevention of neonatal HSV infection can be achieved by preventing transmission of herpes simplex from infected mothers to their infants during delivery.
In addition, the American College of Obstetricians and Gynecologists recommends herpes suppressive therapy for these women from 36 weeks gestation to delivery.
Similarly, women with recurrent herpes should receive herpes suppressive therapy from 36 weeks until delivery. Treatment of hearing loss associated with HSV1 or HSV2 infections includes treatment with antiherpetic agents and steroids.
Hearing loss that does not recover following treatment with steroids and antiherpetic agents can be remediated with hearing aids or cochlear implantation, depending on the severity of loss. The measles virus rubeola is an enveloped single-stranded RNA virus in the paramyxovirus family. It is very easily transmitted through contact with respiratory secretions from patients with measles.
Symptoms include fever, cough, nasal congestion, erythematous maculopapular rash, conjunctivitis, and pathognomonic Koplik spots on the buccal mucosa. In , measles was declared eliminated from the United States, although cases resulting from immigration or importation of the virus continue to occur McLean et al. Fortunately, most children recover from measles without long-term side effects. Hearing loss resulting from measles infection is typically bilateral, moderate to profound SNHL and may follow measles encephalitis McKenna, Measles is associated with a high incidence of otitis media in up to 8.
Sequelae of bacterial superinfection may account for some cases of hearing loss associated with measles infection Suboti, Compilation of infection data from multiple countries shows MMR and other anti-measles vaccinations are rarely associated with SNHL, with a time course of onset corresponding to incubation period of measles infection and an incidence of 1 case per 6 to 8 million vaccine doses.
Because MMR vaccine also includes live attenuated mumps virus, it is possible that the measles vaccine is not the sole cause of these cases. SNHL associated with vaccination ranges in severity, can be bilateral or unilateral, and has been reported in both pediatric and adult populations.
In some of these patients, symptoms such as fever, rash, headache, and ataxia have been reported after vaccination and prior to onset of hearing loss, consistent with the possibility that hearing loss is the result of vaccination-induced encephalitis Asatryan et al.
Postexposure prophylaxis is through the use of intramuscularly administered measles immune globulin for infants aged 0 to 6 months and in older immunocompetent people.
In pregnant woman without evidence of measles immunity and in severely immunocompromised patients, the measles immune globulin should be administered intravenously McLean et al. Treatment for measles infection is supportive, including intravenous fluids for dehydration and diarrhea. Secondary infections, including pneumonia, can occur and should be treated promptly Sabella, Treatment of hearing loss resulting from measles depends on the degree and type of hearing loss noted.
Measles infection has been hypothesized to cause otosclerosis, which causes stapes fixation and subsequent CHL as well as SNHL due to the formation of abnormal foci of bone remodeling in the middle and inner ear.
Patients with otosclerosis can be treated with either hearing amplification or stapedotomy for their conductive loss and with hearing aids for SNHL. For patients with severe SNHL from otosclerosis, cochlear implantation or stapedotomy for far advanced otosclerosis plus a postoperative hearing aid can rehabilitate hearing Semaan et al. VZV is a highly contagious virus that is transmitted either by droplets from coughing or sneezing of actively infected individuals or by direct contact with fluid from herpetic vesicles.
VZV-infected patients are infectious from 2 days prior to appearance of the viral rash until after all the vesicles have crusted. VZV first causes a primary infection that, when symptomatic, manifests with fever, an erythematous macular rash, and pustules chickenpox. In some individuals, the primary infection is asymptomatic. The virus can subsequently remain latent in neurons in various parts of the body for an extended period, reactivating years later.
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