“Reimagining Pain Management: SZM Nerve Block in Pediatric Adenotonsillectomy”Dear Editor! With approximately 289,000 procedures performed in 2010 alone, adenotonsillectomy is one of the most common surgeries among the pediatric patient population (1). Despite its reputation for favorable perioperative outcomes, a substantial body of research indicates that adenotonsillectomy is also one of the most painful pediatric surgeries (2). The current insufficient and potentially unsafe treatment options for managing postoperative pain after adenotonsillectomy compel patients to resort to high doses of opioids, posing long-term adverse effects. In this critical context, suprazygomatic maxillary (SZM) nerve block, an intricate anesthetic technique, emerges as a promising solution.An SZM nerve block is a regional anesthesia technique predominantly employed to provide analgesia for surgeries involving the midface, such as procedures on the maxillary sinus, upper lip, and upper teeth. This anesthetic nerve block targets the maxillary nerve (V2), which is the second branch of the trigeminal nerve (cranial nerve V).A recently published randomized clinical trial in JAMA Otolaryngology-Head & Neck Surgery evaluated the SZM nerve block for patients undergoing adenotonsillectomy. This first-of-its kind, groundbreaking trial demonstrated that SZM block was effective in minimizing postoperative pain and significantly reducing postoperative opioid consumption (3). Furthermore, the SZM nerve block also had a higher opioid-free postanesthesia care unit (PACU) stay compared with placebo, reflecting its efficacious impact on postoperative opioid utilization (3). These findings are corroborated by a case report by Smith et al., which showed clinically meaningful analgesic benefit from SZM nerve block for adenoidectomy and tonsillectomy, as the patient did not require any postoperative opioid drugs (4).Opioids remain a major part of postoperative pain management for pediatric patients after adenotonsillectomy. However, the black box warning and contraindication statements from the US Food and Drug Administration stress that opioids are known to result in serious postoperative consequences, including respiratory depression and even death (5). The SZM nerve block offers a highly effective and safe alternative for managing postoperative pain in these patients. To fully establish if the SZM nerve block is the silver bullet for opioid-free postoperative pain management, more clinical trials with larger patient populations are required. Adopting this technique could address the urgent need for safer pain management methods in pediatric surgery, as well as improve recovery outcomes and patient safety.Keywords: adenotonsillectomy, suprazygomatic maxillary nerve block, opioids, analgesiaDisclaimer: None to declareConflict of Interest: None to declareFunding Disclosure: None to declareREFERENCESHall MJ, Schwartzman A, Zhang J, Liu X. Ambulatory Surgery Data From Hospitals and Ambulatory Surgery Centers: United States, 2010. Natl Health Stat Report. 2017 Feb;(102):1-15. PMID: 28256998.Persino PR, Saleh L, Walner DL. Pain control following tonsillectomy in children: A survey of patients. Int J Pediatr Otorhinolaryngol. 2017 Dec;103:76-79. doi: 10.1016/j.ijporl.2017.10.016. Epub 2017 Oct 7. PMID: 29224770.Lin C, Abboud S, Zoghbi V, Kasimova K, Thein J, Meister KD, Sidell DR, Balakrishnan K, Tsui BCH. Suprazygomatic Maxillary Nerve Blocks and Opioid Requirements in Pediatric Adenotonsillectomy: A Randomized Clinical Trial. JAMA Otolaryngol Head Neck Surg. 2024 May 23:e241011. doi: 10.1001/jamaoto.2024.1011. Epub ahead of print. PMID: 38780948; PMCID: PMC11117150.Smith L, Balakrishnan K, Pan S, Tsui BCH. Suprazygomatic maxillary (SZM) nerve blocks for perioperative pain control in pediatric tonsillectomy and adenoidectomy. J Clin Anesth. 2021 Aug;71:110240. doi: 10.1016/j.jclinane.2021.110240. Epub 2021 Mar 20. PMID: 33756446.FDA updates safety review of codeine use in children; new Boxed Warning and Contraindication on use after tonsillectomy and/or adenoidectomy. FDA; [cited 2024 Jun 3]. Available from: https://www.fda.gov/science-research/pediatrics/fda-pediatric-safety-communications

Is Gene Therapy the Game-Changer for Autosomal Recessive Deafness 9?Dear editor! One of the most challenging forms of hearing impairment is autosomal recessive profound prelingual deafness (DFNB9), which makes up over 80% of all congenitally occurring hearing loss cases (1). Otoferlin protein, encoded by the OTOF gene, is located at the inner hair cells, which mediates signal transduction for proper sound propagation. The OTOF gene mutations impair the physiological otoferlin function and account for almost 1–8% of all the DFNB9 cases (2). Currently, there are no medical therapies available to combat this disorder, and in this critical context, adeno-associated virus (AAV) serotype 1 carrying a human OTOF transgene (AAV1-hOTOF) may offer promising results.AAV1-hOTOF is an experimental gene therapy intended to treat DFNB9. It transports the functional copy of the OTOF gene to the hair cells via an adenovirus vector. This enables the hair cells to produce newly functioning otoferlin protein, thereby regaining proper signal transduction and sound propagation.A groundbreaking clinical trial, recently published in The Lancet, evaluates the efficacy and safety of this AAV1-hOTOF gene therapy for restoring hearing in patients with DFNB9. This first-of-its-kind trial demonstrated that AAV1-hOTOF showed excellent results and helped in reclaiming significant functional hearing in all the patients enrolled in the trial (3). Furthermore, along with enhanced hearing, gene therapy also assisted in improving speech perception in the patients. In addition to its increased efficacy, gene therapy had a favorable safety profile, with no reports of serious adverse events or drug-limiting ototoxicity (3). These findings of a successful response to gene therapy are in line with the case report by Qi et al., which also showed that AAV1-hOTOF helped restore substantial hearing in two children (4).In South Asia, 1.62 out of every 1000 newborns suffer from congenital hearing loss, making it one of the most prevalent chronic pediatric illnesses (5). Due to the unavailability of any medical intervention, DFNB9 goes untreated, substantially affecting the quality of life of patients. However, with the introduction of gene therapies like AAV1-hOTOF, there is newfound hope for these patients. This pioneering therapy may completely eradicate DFNB9 and significantly enhance auditory function, providing a viable substitute for cochlear implants and conventional hearing aids. More clinical trials assessing larger patient populations are warranted to provide greater evidence for the effectiveness and tolerability of this therapy.Keywords: autosomal recessive deafness 9; AAV1-hOTOF; congenital hearing loss; AsiaDisclaimer: None to declareConflict of Interest: None to declareFunding Disclosure: None to declareREFERENCESDuman D, Tekin M. Autosomal recessive nonsyndromic deafness genes: a review. Front Biosci (Landmark Ed). 2012 Jun 1;17(6):2213-36. doi: 10.2741/4046. PMID: 22652773; PMCID: PMC3683827.Azaiez H, Thorpe RK, Smith RJH. OTOF -Related Deafness. 2008 Feb 29 [updated 2021 Jan 21]. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Bean LJH, Gripp KW, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2024. PMID: 20301429.Lv J, Wang H, Cheng X, Chen Y, Wang D, Zhang L, Cao Q, Tang H, Hu S, Gao K, Xun M, Wang J, Wang Z, Zhu B, Cui C, Gao Z, Guo L, Yu S, Jiang L, Yin Y, Zhang J, Chen B, Wang W, Chai R, Chen ZY, Li H, Shu Y. AAV1-hOTOF gene therapy for autosomal recessive deafness 9: a single-arm trial. Lancet. 2024 May 25;403(10441):2317-2325. doi: 10.1016/S0140-6736(23)02874-X. Epub 2024 Jan 24. Erratum in: Lancet. 2024 May 25;403(10441):2292. PMID: 38280389.Qi J, Tan F, Zhang L, Lu L, Zhang S, Zhai Y, Lu Y, Qian X, Dong W, Zhou Y, Zhang Z, Yang X, Jiang L, Yu C, Liu J, Chen T, Wu L, Tan C, Sun S, Song H, Shu Y, Xu L, Gao X, Li H, Chai R. AAV-Mediated Gene Therapy Restores Hearing in Patients with DFNB9 Deafness. Adv Sci (Weinh). 2024 Mar;11(11):e2306788. doi: 10.1002/advs.202306788. Epub 2024 Jan 8. PMID: 38189623; PMCID: PMC10953563.Korver AM, Smith RJ, Van Camp G, Schleiss MR, Bitner-Glindzicz MA, Lustig LR, Usami SI, Boudewyns AN. Congenital hearing loss. Nat Rev Dis Primers. 2017 Jan 12;3:16094. doi: 10.1038/nrdp.2016.94. PMID: 28079113; PMCID: PMC5675031.