Hearing Losses

Hearing loss can be caused by a variety of reasons. It can be unilateral, bilateral, progressive, fluctuating.

Different reasons can cause hearing loss in children and adults. With hearing loss, ear fullness, pain, discharge, dizziness, tinnitus, cranial nerve involvement can be seen.

Acute hearing loss may be due to sudden idiopathic hearing loss, viral infection, bacterial infection, abnormalities in semicircular canal fluid, vascular problems, trauma, tumors, autoimmune, nervous system problems, medication side effects.

Mumps, measles, rubella and Epstein-Bar (EBV) virus can directly affect the ear and cause temporary or permanent hearing loss in varying degrees. In these cases, dizziness may or may not accompany the condition. Again, these infections can cause neural-type hearing loss with a toxic effect on the labyrinth.

Ramsay-Hunt syndrome typically affects the facial nerve and auditory-balance nerve by involving the nerve and causes hearing loss.

Bacterial or fungal meningitis causes sudden hearing loss by negatively affecting the labyrinth. The rate of neural-type hearing loss in children after bacterial meningitis is between 3.5% and 37%.

Acute otitis media, chronic otitis media affecting the labyrinth, and syphilis (gonorrhea) are other infectious causes of sudden hearing loss.

Abnormalities of the semicircular canal, such as increased pressure of fluid in the labyrinth (semicircular canals) (hydrops) or perilymphatic fistula (leakage of fluid in the semicircular canal through an opening), can cause permanent sudden hearing loss that rises and falls. Heavy lifting, head injury, or barotrauma can cause fluid in the semicircular canal to leak out through an opening (perilymphatic fistula). Increased pressure of the semicircular canal fluid (hydrops) typically causes hearing loss at low frequencies.

Vascular events involving the cochlea (snail) may cause sudden hearing loss. Thrombosis (blood clotting in a vein) or embolism (clotted blood is carried through the vein to occlude smaller vessels) of the labyrinth and cochlear arteries is not very common. Hearing loss may occur due to decreased blood supply of the cochlea in Polycystemia Vera disease where the blood viscosity is decreased. Diabetes in which small vessels are blocked, arteriosclerosis, sickle cell anemia are other causes of hearing loss.

Head trauma, barotrauma and loud noise can cause temporary and permanent acute hearing loss. Conduction or neural-type hearing loss can be seen in temporal bone (the bone in which the ear is located) fractures due to direct head trauma. Concussion-induced damage to the labyrinth membrane can also cause hearing loss, even without a fracture of the temporal bone.

The cerebellopontine corner can be defined as the corner between the cerebellum and the spinal cord. Tumors in this area can cause sudden, temporary or permanent hearing loss.

Autoimmune diseases may be associated with neural-type hearing loss (relapsing polychondritis, polyarteritis nodosa, sarcoidosis, Crohn’s disease, Cogan’s syndrome, systemic lupus erythematosus). Typically, hearing loss is not the first finding in these diseases, and there are other accompanying signs and symptoms in the history and physical examination. Hearing loss in these diseases; It may start unilaterally and be bilateral, it may be in the form of sudden neural-type hearing loss, and it may be a fluctuating type of hearing loss.

While neurologic findings accompanying hearing loss indicate Multiple Sclerosis, the presence of visual findings should bring Susac syndrome to mind. Susac syndrome is a syndrome in which occlusion of the vessel feeding the layer behind the eye (retinal artery), encephalopathy and cochlear microangiopathy (impairment of the snail’s blood supply) are seen together.

Causes of bilateral, slowly progressive hearing loss in adults

Presbycusis (aging-related hearing loss), exposure to loud noise, or familial neural-type hearing loss may occur. If there is hearing loss if it is unilateral and neural type, retrocochlear pathology should be investigated. If there is a conductive component in unilateral hearing loss, the reason is; Conditions such as otosclerosis, chronic otitis media with effusion, loss of mobility in the head of the malleus (anvil), tympanosclerosis (calcification in the middle ear elements), myringosclerosis (calcification in the eardrum), tympanic membrane hole, cholesteatoma and loss of continuity in the ossicular chain should be considered.

Masses originating from the jigular foramen (the hole through which the main vein passes at the base of the skull) may cause conductive hearing loss with the mass effect, or they may cause neural-type hearing loss with melting of the cochlea and direct compression on the auditory nerve.

Menire’s disease, perilymphatic fistula, multiple sclerosis, tuberculosis, autoimmune diseases and sarcoidosis cause fluctuating hearing loss. In Menire’s disease, there are findings related to balance with hearing loss. However, syphilitic (inner ear effects of the microorganism that causes gonorrhea) inner ear disease and perilymphatic fistula can be difficult to diagnose because there are variable findings. Since there are other accompanying systemic findings in autoimmune diseases, the diagnosis can be reached more easily.

Rapidly progressive and sudden hearing loss can be observed as a result of the spread of tumors of the brain and organs distant to the temporal bone. In this case, other signs and symptoms such as tinnitus, dizziness, balance disorder, and facial paralysis may be added to hearing loss. Tumors of the breast, lung, kidney, stomach, bronchi, and prostate can potentially spread to the temporal bone, skull base, and brain, causing hearing loss.

If children have hearing loss, many factors must be considered.

What are the risk factors for hearing loss in newborns?

  • Childhood in the family genetic hearing lossto be a person

  • Cytomegalovirus, rubella, syphilis, herpes, and toxoplasma infection at the end of pregnancy and at birth,

  • Craniofacial (craniofacial and facial bones) anomalies,

  • Birth weight below 1500 g,

  • Neonatal jaundice requiring exchange transfusion,

  • Treatment with drugs that can harm the ear,

  • History of bacterial meningitis,

  • Apgar score in the range of 0-4 at the 1st minute or 0-6 at the 5th minute,

  • It can be listed as long-term postpartum stay on the respirator.

  • Biotinidase deficiency is a genetic (autosomal recessive) disease caused by a defect in the recycling mechanism of biotin vitamin. In this disease, which is characterized by mental retardation, hypotonia, epileptic seizures, baldness and skin rash, neural-type hearing loss is observed at a rate of 75%. Early diagnosis in the neonatal period is important for treatment.

  • Exposure to infection in the womb and drug use can also cause hearing loss in the baby.

Syndromes that cause congenital and genetic hearing loss.

  • Oculoauriculovertebral spectrum,

  • sticker syndrome,

  • congenital cytomegalovirus,

  • Usher’s syndrome,

  • Branchio-otorenal syndrome,

  • pendredS syndrome,

  • CHARGE association,

  • neurofibromatosis type II,

  • Waardenburg’s syndrome,

  • Mitochondrial diseases,

  • otopalatal-digital syndrome,

  • osteogenesis imperfecta,

  • metabolic storage diseases,

  • Townes-Brock syndrome,

  • Wildervanc syndrome,

  • can be listed as biotinidase deficiency.

The degree of hearing loss in these syndromes varies widely. There is hearing loss that increases with the magnitude of the anomaly.

Another developmental anomaly that is very rare in children and causes conductive hearing loss is salivary gland choristoma. He has conductive hearing loss and a mass in the middle ear. This middle ear mass is often attached to the part of the facial nerve that passes through the middle ear. In addition, there are middle ear ossicular chain disorder and outer ear anomaly.

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