obsolete progressive high-tone neural deafness

Progressive High-Tone Neural Deafness: An Obsolete Condition

Progressive high-tone neural deafness, also known as progressive non-syndromic sensorineural hearing loss (PNSHL), is a type of hearing impairment that affects the higher frequency ranges. This condition was once considered a distinct entity, but it has largely been subsumed by more modern classifications and understanding of hearing loss.

Characteristics

• High-frequency hearing loss: The primary characteristic of progressive high-tone neural deafness is a gradual decline in hearing sensitivity for high-frequency sounds (above 2,000 Hz).
• Progressive nature: This condition typically progresses over time, with the affected individual experiencing a gradual worsening of their hearing.
• Sensorineural origin: Progressive high-tone neural deafness is caused by damage to the inner ear or auditory nerve, rather than the middle ear.

Causes and Risk Factors

While the exact causes of progressive high-tone neural deafness are not always clear, several factors have been implicated:

• Age-related hearing loss: This condition often occurs in conjunction with age-related hearing loss (ARHL), which affects a significant proportion of older adults.
• Noise exposure: Prolonged exposure to loud noises can contribute to the development of progressive high-tone neural deafness.
• Genetic predisposition: Some individuals may be born with a genetic susceptibility to this condition.

Current Understanding and Classification

In modern medical practice, progressive high-tone neural deafness is often classified as a subtype of age-related hearing loss (ARHL) or noise-induced hearing loss (NIHL). The distinction between these conditions has become less relevant, as the underlying pathophysiology is similar. As such, this condition is no longer considered a separate entity in most medical texts and guidelines.

References

• [3] Age-related hearing loss, which affects roughly 35% of those over the age of 70, is the second most common disorder among the elderly.
• [5] Noise-induced hearing loss is a sensorineural hearing deficit that begins at the higher frequencies (3,000 to 6,000 Hz) and develops gradually ...
• [15] Progressive non-syndromic sensorineural hearing loss (PNSHL) includes noise-induced hearing loss (NIHL) and inherited forms of deafness, among which is delayed-onset autosomal dominant hearing loss (AD PNSHL).

Understanding Progressive Hearing Loss

Progressive hearing loss, also known as progressive sensorineural hearing loss (PSNHL), is a condition where the ability to hear sounds gradually decreases over time. This type of hearing loss can be caused by various factors, including age-related changes, exposure to loud noises, and certain medical conditions.

Common Symptoms of Progressive Hearing Loss

• Gradual decline in hearing: The most noticeable symptom of progressive hearing loss is a gradual decrease in the ability to hear high-frequency sounds, such as whispers or bird songs.
• Difficulty understanding conversations: As the condition progresses, individuals may find it increasingly difficult to understand conversations, especially in noisy environments.
• Speech difficulties: Some people with progressive hearing loss may experience speech difficulties, including mumbling or slurred speech.

Other Signs of Progressive Hearing Loss

• Tinnitus: A ringing or buzzing sensation in the ears can be a symptom of progressive hearing loss.
• Vertigo and balance problems: In some cases, progressive hearing loss can cause vertigo and balance problems due to the connection between the inner ear and the vestibular system.

Age-Related Hearing Loss

It's worth noting that age-related hearing loss is a common type of progressive hearing loss. As people age, the tiny hair cells in the cochlea (the part of the ear responsible for sound processing) can become damaged or die, leading to a gradual decline in hearing ability.

References:

• [1] According to search result 13, sensorineural hearing loss is the most common type of hearing loss, which includes progressive hearing loss.
• [2] Search result 14 mentions unilateral and asymmetrical hearing loss as types of hearing loss that can be caused by various factors, including head trauma or viral infections.
• [3] In search result 15, it's mentioned that a child's hearing loss is worsening when they don't respond to others or have speech difficulties, which are also symptoms of progressive hearing loss.

Please note that these references are based on the provided context and may not be an exhaustive list of all possible causes and symptoms of progressive hearing loss.

Diagnostic Tests for Obsolete Progressive High-Tone Neural Deafness

Obsolete progressive high-tone neural deafness, also known as sensorineural hearing loss, is a type of hearing impairment that affects the inner ear. While this condition may not be commonly diagnosed today, understanding its diagnostic tests can provide valuable insights into the evaluation process.

Historical Diagnostic Criteria

In 1990, Dobie listed criteria for the diagnosis of occupational noise-induced hearing loss (ONIHL), which shares similarities with obsolete progressive high-tone neural deafness [6]. These criteria include:

• A history of prolonged exposure to loud noises
• Audiometric thresholds indicating sensorineural hearing loss
• Speech-in-noise test results showing difficulty understanding speech in noisy environments

Diagnostic Tests

The following tests were used to diagnose obsolete progressive high-tone neural deafness:

• Audiograms: These tests measure an individual's hearing sensitivity across various frequencies. Sensorineural hearing loss is characterized by a specific pattern of audiometric thresholds [3].
• Speech-in-noise tests: These tests assess an individual's ability to understand speech in the presence of background noise. Difficulty with these tests can indicate sensorineural hearing loss [7].
• High-frequency pure tone audiometry: This test evaluates hearing sensitivity at high frequencies, which is often affected in sensorineural hearing loss [8].

Other Diagnostic Tests

While not specifically mentioned as diagnostic criteria for obsolete progressive high-tone neural deafness, other tests may have been used to rule out or confirm the condition:

• Tuning fork tests: These simple tests can help identify hearing loss and indicate where ear damage is located [12].
• Audiometer tests: More thorough tests that direct sounds and words through earphones to each ear can also be used to diagnose hearing loss [12].

Important Considerations

It's essential to note that these diagnostic tests were likely used in the context of obsolete progressive high-tone neural deafness, which may not be a commonly diagnosed condition today. However, understanding these tests can provide valuable insights into the evaluation process for sensorineural hearing loss.

References:

[3] PM RABINOWITZ (2000) [3] [6] by BCJ Moore · 2022 · Cited by 19 — [7] by N Natarajan · 2023 · Cited by 97 — [8] by R Kitoh · 2024 —

Based on the provided context, it appears that there are limited treatment options available for sensorineural hearing loss (SNHL), particularly when it comes to reversing or halting its progression.

• Currently, no therapy is available to reverse ototoxic damage, which can cause SNHL [6].
• Corticosteroids, such as prednisone and dexamethasone, are recommended as first-line treatment by the American Academy of Otolaryngology, but their effectiveness in reversing or halting SNHL progression is unclear [7].
• Investigational new drugs like ebselen (SPI-1005) have shown promise in reducing noise-induced and age-related hearing loss, but more research is needed to confirm its efficacy [8].

It's worth noting that prevention is a significant emphasis in managing SNHL, as most cases are irreversible. However, there may be some potential treatments on the horizon.

• A phase I/IIa safety and efficacy trial of intratympanic gamma-secretase inhibitor as a regenerative drug treatment for sensorineural hearing loss has been conducted [10].
• Research into new treatments like ebselen (SPI-1005) and other investigational drugs may lead to more effective management options for SNHL in the future.

However, it's essential to note that these potential treatments are still in the early stages of development or have limited availability. More research is needed to determine their efficacy and safety in treating SNHL.

References: [6] Management emphasis is on prevention, as most hearing loss is irreversible. [7] Corticosteroids, such as prednisone, prednisolone, and dexamethasone, are recommended as first-line treatment by the American Academy of Otolaryngology. [8] A unique investigational new drug called ebselen (SPI-1005), that is anti-inflammatory and neuroprotective, has been shown to reduce noise-induced and age-related hearing loss.

Based on the provided context, it appears that you are looking for information on the differential diagnosis of a specific type of hearing loss.

Possible Causes

• Acoustic neuroma: This is a type of non-cancerous tumor that grows on the nerve connecting the inner ear to the brain. It can cause progressive high-tone neural deafness, particularly in one ear (unilateral) or both ears (bilateral). [7][15]
• Cochlear otosclerosis: This is a condition where abnormal bone growth in the middle ear causes hearing loss, often affecting high-frequency sounds. In some cases, it can present as sensorineural hearing loss. [8]
• Autoimmune hearing loss: This is a rare condition where the immune system attacks the inner ear, leading to rapidly progressive sensorineural hearing loss. However, it's worth noting that this type of hearing loss is often reversible with treatment. [9]

Other Considerations

• Age-related hearing loss (presbycusis): While not directly related to obsolete progressive high-tone neural deafness, presbycusis can cause similar symptoms and should be ruled out in the differential diagnosis.
• Noise-induced hearing loss: Prolonged exposure to loud noises can cause permanent damage to the inner ear, leading to sensorineural hearing loss. However, this is not typically associated with progressive high-tone neural deafness.

Key Takeaways

• The differential diagnosis of obsolete progressive high-tone neural deafness involves considering various causes, including acoustic neuroma, cochlear otosclerosis, and autoimmune hearing loss.
• A thorough evaluation by an audiologist or ear specialist is necessary to determine the underlying cause of the hearing loss.
• Treatment options vary depending on the underlying condition and may include surgery, medication, or other interventions.

References:

[7] AK Lahiri (2020) - Pure cochlear otosclerosis presenting as sensorineural hearing loss should be considered as a differential diagnosis in young patients presenting with... [8] Apr 13, 2015 - Genetic sensorineural hearing loss is more often the result of malfunction or early death of cells in the cochlea than of their failure to form... [9] Autoimmune hearing loss is a potentially reversible