Introduction

Congenital deafness significantly impacts speech, language and cognitive development. Hearing loss being a hidden disability, is often detected late. Critical developmental plasticity of brain happens by 5 years of age. Inability to diagnose and intervene in an early stage of life leads to permanent language disability in children. Cochlear implantation has revolutionized the management of profound sensorineural hearing loss, especially when performed early in life. Early auditory stimulation is crucial for neural plasticity and optimal rehabilitation outcomes.

Case presentation

A 19-month-old child presented with a history of lack of response to auditory stimuli since birth. There was no significant prenatal, perinatal, or postnatal complication reported. Developmental milestones were appropriate for age except for speech and auditory responses.

Clinical evaluation

No response to even loud sound stimuli
Absent startle reflex to loud sounds
Delayed speech milestones

Pre-operative audiological assessment

Otoacoustic emissions (OAE): Absent bilaterally
Brainstem evoked response audiometry (BERA): Findings consistent with bilateral profound sensorineural hearing loss

Psychological assessment

On developmental screening test, the child obtained a DQ of 88, while Vineland social maturity scale yielded an SQ of 110.

Radiological assessment

Pre-operative imaging (HRCT temporal bone & MRI brain) revealed normal cochlear anatomy with patent cochlear turns and normal cochlear nerve bundle, making the child a suitable candidate for cochlear implantation.

On Otoplan, a cochlear duct length of 31.3 mm was calculated and hence flex 28 electrode was deemed suitable.

Intervention

The child underwent unilateral cochlear implantation under general anaesthesia. Standard posterior tympanotomy approach was used. MED-EL Sonata 2 implant with Flex 28 electrode array was successfully inserted into the scala tympani without resistance.

Intraoperative findings

Proper placement of receiver-stimulator and full electrode insertion achieved.
The position was confirmed by intraoperative C-arm.
Intraoperative Neural Response Telemetry confirmed functional status of all the 12 electrodes in the cochlea.
No intraoperative complications seen.

Postoperative course

The postoperative period was uneventful. The wound healed well with no signs of infection or complications.

Device activation (switch-on)

At 3 weeks post-surgery, the cochlear implant was activated. On switch-on, the child demonstrated immediate behavioural responses to sound, indicating successful auditory perception for the first time in life. This marked a crucial milestone in auditory rehabilitation.

The takeaways

Early cochlear implantation, especially before the age of 2 years, is associated with superior auditory and speech outcomes due to enhanced neural plasticity.
Early diagnosis through newborn hearing screening.
Timely surgical intervention.
Objective intraoperative measures like NRT.
Multidisciplinary teamwork including cochlear implant surgeon, anaesthetists, audiologists, nursing staff, and OT technicians

The intraoperative imaging (C-arm) and telemetry findings in this case confirmed accurate electrode placement and good neural responsiveness, which are predictive of favourable postoperative outcomes.

Conclusion

This case demonstrates that early cochlear implantation can successfully provide auditory access to children born with profound hearing loss. The first auditory experience post-activation is a pivotal milestone and underscores the life-changing impact of this intervention. Continued auditory-verbal therapy and structured follow-up will be essential for optimal speech and language development.

Acknowledgement

We sincerely acknowledge the contributions of the anaesthesia team, nursing staff, OT technicians, and hospital authorities for their support in the successful management of this case.