Hair Cells

Understanding hair cells: definition

Hair cells are sensory cells found in the auditory and vestibular systems, named due to the filamentous structures called stereocilia located at their apex. In the cochlea, two types of hair cells are identified: inner hair cells (IHCs) and outer hair cells (OHCs). Humans have approximately 3,500 inner hair cells, with their size gradually increasing towards the apex of the cochlea.

Function and role of hair cells

The outer hair cells, arranged in three rows, number approximately 12,500 and are cylindrical in shape. The apical surface of these cells and their stereocilia are immersed in endolymph. The sensory or auditory cells, supporting cells, basilar membrane, spiral groove, and tectorial membrane together form the organ of Corti.

Hair cells are responsible for converting the hydromechanical energy transferred to the cochlea by the movements of the eardrum and ossicle chain into bioelectric signals. These signals are then transmitted via the auditory nerve fibres to the auditory regions of the brain. Within the cochlea, hair cells are organised in rows of varying sizes, each corresponding to different frequencies in the sound spectrum that humans are capable of hearing.

The structure of the hair cells, along with the unique properties of their cilia, enables them to detect minute displacements on the nanometre scale, which can occur in response to even very low-intensity acoustic stimuli. Although outer hair cells vastly outnumber inner hair cells, 95% of auditory nerve fibres innervate the inner hair cells, with only 5% connecting to the outer hair cells.

Structure and function of external and internal hair cells

External hair cells

The outer hair cells, numbering approximately 12,500, exhibit a highly regular cylindrical shape and have 100 to 150 stereocilia arranged in three rows per cell. Outer hair cells have minimal sensory function, with their innervation being almost entirely efferent. Their role is to provide a motor function that fine-tunes the cochlear mechanics in response to sound waveforms, playing a crucial role in frequency selectivity—distinguishing between different frequencies perceived simultaneously. Thanks to the presence of stereocilia and prestin, a protein that enables the outer hair cells to alter their length in response to sound signals, outer hair cells act as amplifiers of acoustic signals.

Internal hair cells

The inner hair cells serve as the primary vibration sensors of the basilar membrane. Their depolarisation activates the afferent nerve fibres, allowing them to transmit sound information to the auditory nerve fibres and, subsequently, to the central nervous system.

The depolarisation of inner hair cells is directly proportional to both the intensity of the sound stimulus and the amplification provided by the motility of the outer hair cells. Inner hair cells are generally more resilient than outer hair cells, which are more susceptible to damage from noise exposure, ototoxic drugs, and antibiotics.

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The impact of hair cell on hearing loss

Damaged hair cells

At birth, the hair cells in the organ of Corti are present in large numbers, but they cannot regenerate. Various factors can damage these cells, reducing their number and leading to deafness. The most common causes of hearing loss due to damage to cochlear cells include:

  • Exposure to loud noise (workplace environments, loud music, hobbies)
  • Presbycusis (age-related decline in hearing ability)
  • Infections (such as otitis)
  • Congenital deafness
  • Head trauma
  • Cochlear malformation
  • Oxidative stress results in the destruction of hair cells, which are vital for receiving sounds and transmitting them to the auditory regions of the brain. 

Consequently, damage or loss of hair cells in the inner ear can lead to varying degrees of hearing impairment, affecting sound perception and speech comprehension.

Hair cells and sensorineural hearing loss

Hearing loss or deafness may be present from birth and can result from genetic factors (congenital deafness) or complications during pregnancy or birth (acquired deafness). The use of ototoxic drugs during pregnancy, or drug misuse, also poses a high risk of the unborn child developing deafness.

In adulthood, there are numerous causes of deafness, including infections, ototoxic drugs or substances, acoustic trauma, and presbycusis. Depending on the part of the ear affected, hearing loss is classified as either conductive or sensorineural. Conductive hearing loss occurs when damage affects the outer or middle ear, while sensorineural hearing loss results from damage to the inner ear or auditory nerve.

When the number of hair cells diminishes, sensorineural hearing loss develops. Due to their vital role in the sound transmission process, the loss of hair cells leads to permanent hearing impairment. In 90% of cases of hearing loss or acquired deafness, the condition is caused by the death of hair cells in the organ of Corti.

Can hair cells regenerate themselves?

Hair cells do not naturally regenerate. However, research into pharmaceutical treatments for deafness is advancing, with one promising area being the regeneration of hair cells in the inner ear. Scientists at the Massachusetts Eye and Ear Infirmary (USA) have discovered that it is possible to reactivate the cellular mechanism that enables supporting cells in the organ of Corti to differentiate into hair cells. This is achieved by reactivating two proteins, MYC and NOTCH1. These interventions result in the proliferation of cells with certain characteristics of hair cells, such as transduction channels and the ability to connect with spiral ganglion neurons.

Can hair cells aid in hearing restoration?

The Belgian researcher Brigitte Malgrande and her team in Liège have been exploring this concept for several years: modifying other cells in the organ of Corti without impacting the overall population of auditory cells. The Ephrin-B2 protein is exclusively found in supporting cells; when this protein is rendered inactive, the supporting cell transforms into a hair cell. Current research is focused on establishing connections between these newly formed hair cells and neurons, which is crucial for transmitting external sounds to the brain. This research is being conducted at GIGA-Neurosciences. If the researchers succeed in 'creating' hair cells and connecting them to the neurons, it could lead to a partial restoration of impaired hearing.

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Best hearing aids for sensorineural hearing loss

Until cochlear cell regeneration becomes a possibility, the only solution for sensorineural hearing loss resulting from hair cell damage is the use of hearing aids.

Hearing aids

Hearing aids are the most common and effective solution, particularly in cases of hearing loss. Amplifon provides cutting-edge hearing aids that ensure improved speech comprehension, listening comfort, and convenience in daily life.

We strongly recommend that individuals with sensorineural hearing loss acquire hearing aids as early as possible to maintain their ability to understand speech. Early fitting of a hearing aid ensures the best outcomes over time and helps to mitigate various sensory alterations, such as cognitive decline, which often accompany deafness.

Cochlear implant

In cases of profound hearing loss that cannot be addressed with hearing aids, a cochlear implant, which is surgically implanted, is the necessary solution to compensate for the hearing impairment. It is always advisable to consult your doctor or ENT specialist for further information, which can also be obtained from Amplifon hearing care professionals.
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